Autoantibodies directed against cytoplasmic constituents of POLYMORPHONUCLEAR LEUKOCYTES and/or MONOCYTES. They are used as specific markers for GRANULOMATOSIS WITH POLYANGIITIS and other diseases, though their pathophysiological role is not clear. ANCA are routinely detected by indirect immunofluorescence with three different patterns: c-ANCA (cytoplasmic), p-ANCA (perinuclear), and atypical ANCA.
Inflammation of any one of the blood vessels, including the ARTERIES; VEINS; and rest of the vasculature system in the body.
A polymorphonuclear leukocyte-derived serine protease that degrades proteins such as ELASTIN; FIBRONECTIN; LAMININ; VITRONECTIN; and COLLAGEN. It is named for its ability to control myeloid cell growth and differentiation.
A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (VASCULITIS) leading to damage in any number of organs. The common features include granulomatous inflammation of the RESPIRATORY TRACT and kidneys. Most patients have measurable autoantibodies (ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES) against neutrophil proteinase-3 (WEGENER AUTOANTIGEN).
Group of systemic vasculitis with a strong association with ANCA. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls.
Granular leukocytes having a nucleus with three to five lobes connected by slender threads of chromatin, and cytoplasm containing fine inconspicuous granules and stainable by neutral dyes.
Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).
A hemeprotein from leukocytes. Deficiency of this enzyme leads to a hereditary disorder coupled with disseminated moniliasis. It catalyzes the conversion of a donor and peroxide to an oxidized donor and water. EC 1.11.1.7.
A primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls.
Widespread necrotizing angiitis with granulomas. Pulmonary involvement is frequent. Asthma or other respiratory infection may precede evidence of vasculitis. Eosinophilia and lung involvement differentiate this disease from POLYARTERITIS NODOSA.
A form of necrotizing non-granulomatous inflammation occurring primarily in medium-sized ARTERIES, often with microaneurysms. It is characterized by muscle, joint, and abdominal pain resulting from arterial infarction and scarring in affected organs. Polyarteritis nodosa with lung involvement is called CHURG-STRAUSS SYNDROME.
A heterogeneous group of diseases characterized by inflammation and necrosis of the blood vessel walls.
The property of antibodies which enables them to react with some ANTIGENIC DETERMINANTS and not with others. Specificity is dependent on chemical composition, physical forces, and molecular structure at the binding site.
Inflammation of the retinal vasculature with various causes including infectious disease; LUPUS ERYTHEMATOSUS, SYSTEMIC; MULTIPLE SCLEROSIS; BEHCET SYNDROME; and CHORIORETINITIS.
Inflammation of blood vessels within the central nervous system. Primary vasculitis is usually caused by autoimmune or idiopathic factors, while secondary vasculitis is caused by existing disease process. Clinical manifestations are highly variable but include HEADACHE; SEIZURES; behavioral alterations; INTRACRANIAL HEMORRHAGES; TRANSIENT ISCHEMIC ATTACK; and BRAIN INFARCTION. (From Adams et al., Principles of Neurology, 6th ed, pp856-61)
Disorder characterized by a vasculitic syndrome associated with exposure to an antigen such as a drug, infectious agent, or other foreign or endogenous substance. Its pathophysiology includes immune complex deposition and a wide range of skin lesions. Hypersensitivity or allergy is present in some but not all cases.
Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.
Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY.
A form of fluorescent antibody technique commonly used to detect serum antibodies and immune complexes in tissues and microorganisms in specimens from patients with infectious diseases. The technique involves formation of an antigen-antibody complex which is labeled with fluorescein-conjugated anti-immunoglobulin antibody. (From Bennington, Saunders Dictionary & Encyclopedia of Laboratory Medicine and Technology, 1984)
Antibodies produced by a single clone of cells.
Any member of the group of ENDOPEPTIDASES containing at the active site a serine residue involved in catalysis.
Skin diseases affecting or involving the cutaneous blood vessels and generally manifested as inflammation, swelling, erythema, or necrosis in the affected area.
A thiourea antithyroid agent. Propythiouracil inhibits the synthesis of thyroxine and inhibits the peripheral conversion of throxine to tri-iodothyronine. It is used in the treatment of hyperthyroidism. (From Martindale, The Extra Pharmacopeoia, 30th ed, p534)
Immunoglobulins produced in response to VIRAL ANTIGENS.
Immunoglobulins produced in a response to BACTERIAL ANTIGENS.
An immunoassay utilizing an antibody labeled with an enzyme marker such as horseradish peroxidase. While either the enzyme or the antibody is bound to an immunosorbent substrate, they both retain their biologic activity; the change in enzyme activity as a result of the enzyme-antibody-antigen reaction is proportional to the concentration of the antigen and can be measured spectrophotometrically or with the naked eye. Many variations of the method have been developed.
Agents that are used to treat hyperthyroidism by reducing the excessive production of thyroid hormones.
The diffusion or accumulation of neutrophils in tissues or cells in response to a wide variety of substances released at the sites of inflammatory reactions.
Autoantibodies directed against various nuclear antigens including DNA, RNA, histones, acidic nuclear proteins, or complexes of these molecular elements. Antinuclear antibodies are found in systemic autoimmune diseases including systemic lupus erythematosus, Sjogren's syndrome, scleroderma, polymyositis, and mixed connective tissue disease.
Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.
INFLAMMATION of any ARTERIES.
A serine protease found in the azurophil granules of NEUTROPHILS. It has an enzyme specificity similar to that of chymotrypsin C.
The production of ANTIBODIES by proliferating and differentiated B-LYMPHOCYTES under stimulation by ANTIGENS.
The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.
Antibodies that reduce or abolish some biological activity of a soluble antigen or infectious agent, usually a virus.
Endogenous tissue constituents that have the ability to interact with AUTOANTIBODIES and cause an immune response.
ENDOCARDIUM infection that is usually caused by STREPTOCOCCUS. Subacute infective endocarditis evolves over weeks and months with modest toxicity and rare metastatic infection.
Inflammation of the COLON that is predominantly confined to the MUCOSA. Its major symptoms include DIARRHEA, rectal BLEEDING, the passage of MUCUS, and ABDOMINAL PAIN.
A systemic non-thrombocytopenic purpura caused by HYPERSENSITIVITY VASCULITIS and deposition of IGA-containing IMMUNE COMPLEXES within the blood vessels throughout the body, including those in the kidney (KIDNEY GLOMERULUS). Clinical symptoms include URTICARIA; ERYTHEMA; ARTHRITIS; GASTROINTESTINAL HEMORRHAGE; and renal involvement. Most cases are seen in children after acute upper respiratory infections.
Agents that suppress immune function by one of several mechanisms of action. Classical cytotoxic immunosuppressants act by inhibiting DNA synthesis. Others may act through activation of T-CELLS or by inhibiting the activation of HELPER CELLS. While immunosuppression has been brought about in the past primarily to prevent rejection of transplanted organs, new applications involving mediation of the effects of INTERLEUKINS and other CYTOKINES are emerging.
A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states.
Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides.
Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, environmental exposure and its effects, disease diagnosis, metabolic processes, substance abuse, pregnancy, cell line development, epidemiologic studies, etc.
An iron-binding protein that was originally characterized as a milk protein. It is widely distributed in secretory fluids and is found in the neutrophilic granules of LEUKOCYTES. The N-terminal part of lactoferrin possesses a serine protease which functions to inactivate the TYPE III SECRETION SYSTEM used by bacteria to export virulence proteins for host cell invasion.
A measure of the binding strength between antibody and a simple hapten or antigen determinant. It depends on the closeness of stereochemical fit between antibody combining sites and antigen determinants, on the size of the area of contact between them, and on the distribution of charged and hydrophobic groups. It includes the concept of "avidity," which refers to the strength of the antigen-antibody bond after formation of reversible complexes.
Immunoglobulins produced in a response to FUNGAL ANTIGENS.
A formylated tripeptide originally isolated from bacterial filtrates that is positively chemotactic to polymorphonuclear leucocytes, and causes them to release lysosomal enzymes and become metabolically activated.
Sites on an antigen that interact with specific antibodies.
Precursor of an alkylating nitrogen mustard antineoplastic and immunosuppressive agent that must be activated in the LIVER to form the active aldophosphamide. It has been used in the treatment of LYMPHOMA and LEUKEMIA. Its side effect, ALOPECIA, has been used for defleecing sheep. Cyclophosphamide may also cause sterility, birth defects, mutations, and cancer.
Necrotizing VASCULITIS of small and medium size vessels, developing as a complication in RHEUMATOID ARTHRITIS patients. It is characterized by peripheral vascular lesions, cutaneous ULCERS, peripheral GANGRENE, and MONONEURITIS MULTIPLEX.
Disorders of the nose, general or unspecified.
Removal of plasma and replacement with various fluids, e.g., fresh frozen plasma, plasma protein fractions (PPF), albumin preparations, dextran solutions, saline. Used in treatment of autoimmune diseases, immune complex diseases, diseases of excess plasma factors, and other conditions.
Antibodies which react with the individual structural determinants (idiotopes) on the variable region of other antibodies.
An enzyme that catalyzes the hydrolysis of proteins, including elastin. It cleaves preferentially bonds at the carboxyl side of Ala and Val, with greater specificity for Ala. EC 3.4.21.37.
A PREDNISOLONE derivative with similar anti-inflammatory action.
Antibodies obtained from a single clone of cells grown in mice or rats.
Local surface sites on antibodies which react with antigen determinant sites on antigens (EPITOPES.) They are formed from parts of the variable regions of FAB FRAGMENTS.
Expectoration or spitting of blood originating from any part of the RESPIRATORY TRACT, usually from hemorrhage in the lung parenchyma (PULMONARY ALVEOLI) and the BRONCHIAL ARTERIES.
Pathological processes involving any part of the LUNG.
The movement of leukocytes in response to a chemical concentration gradient or to products formed in an immunologic reaction.
The complex formed by the binding of antigen and antibody molecules. The deposition of large antigen-antibody complexes leading to tissue damage causes IMMUNE COMPLEX DISEASES.
A thioureylene antithyroid agent that inhibits the formation of thyroid hormones by interfering with the incorporation of iodine into tyrosyl residues of thyroglobulin. This is done by interfering with the oxidation of iodide ion and iodotyrosyl groups through inhibition of the peroxidase enzyme.
The process in which the neutrophil is stimulated by diverse substances, resulting in degranulation and/or generation of reactive oxygen products, and culminating in the destruction of invading pathogens. The stimulatory substances, including opsonized particles, immune complexes, and chemotactic factors, bind to specific cell-surface receptors on the neutrophil.
An abundant lysosomal-associated membrane protein that has been found to shuttle between LYSOSOMES; ENDOSOMES; and the PLASMA MEMBRANE. Loss of expression of lysosomal-associated membrane protein 2 is associated with GLYCOGEN STORAGE DISEASE TYPE IIB.
Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.
Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.
A class of immunoglobulin bearing mu chains (IMMUNOGLOBULIN MU-CHAINS). IgM can fix COMPLEMENT. The name comes from its high molecular weight and originally being called a macroglobulin.
A condition characterized by the presence of abnormal quantities of CRYOGLOBULINS in the blood. Upon cold exposure, these abnormal proteins precipitate into the microvasculature leading to restricted blood flow in the exposed areas.
Antibodies reactive with HIV ANTIGENS.
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Refers to any inflammation of the sclera including episcleritis, a benign condition affecting only the episclera, which is generally short-lived and easily treated. Classic scleritis, on the other hand, affects deeper tissue and is characterized by higher rates of visual acuity loss and even mortality, particularly in necrotizing form. Its characteristic symptom is severe and general head pain. Scleritis has also been associated with systemic collagen disease. Etiology is unknown but is thought to involve a local immune response. Treatment is difficult and includes administration of anti-inflammatory and immunosuppressive agents such as corticosteroids. Inflammation of the sclera may also be secondary to inflammation of adjacent tissues, such as the conjunctiva.
Represents 15-20% of the human serum immunoglobulins, mostly as the 4-chain polymer in humans or dimer in other mammals. Secretory IgA (IMMUNOGLOBULIN A, SECRETORY) is the main immunoglobulin in secretions.
A common form of hyperthyroidism with a diffuse hyperplastic GOITER. It is an autoimmune disorder that produces antibodies against the THYROID STIMULATING HORMONE RECEPTOR. These autoantibodies activate the TSH receptor, thereby stimulating the THYROID GLAND and hypersecretion of THYROID HORMONES. These autoantibodies can also affect the eyes (GRAVES OPHTHALMOPATHY) and the skin (Graves dermopathy).
Immunoglobulins induced by antigens specific for tumors other than the normally occurring HISTOCOMPATIBILITY ANTIGENS.
An autoimmune disease of the KIDNEY and the LUNG. It is characterized by the presence of circulating autoantibodies targeting the epitopes in the non-collagenous domains of COLLAGEN TYPE IV in the basement membranes of kidney glomeruli (KIDNEY GLOMERULUS) and lung alveoli (PULMONARY ALVEOLI), and the subsequent destruction of these basement membranes. Clinical features include pulmonary alveolar hemorrhage and glomerulonephritis.
Immunoglobulins produced in a response to PROTOZOAN ANTIGENS.
Agents employed in the preparation of histologic or pathologic specimens for the purpose of maintaining the existing form and structure of all of the constituent elements. Great numbers of different agents are used; some are also decalcifying and hardening agents. They must quickly kill and coagulate living tissue.
A complex systemic syndrome with inflammatory and autoimmune components that affect the skin, fascia, muscle, nerve, blood vessels, lung, and heart. Diagnostic features generally include EOSINOPHILIA, myalgia severe enough to limit usual activities of daily living, and the absence of coexisting infectious, autoimmune or other conditions that may induce eosinophilia. Biopsy of affected tissue reveals a microangiopathy associated with diffuse inflammation involving connective tissue. (From Spitzer et al., J Rheumatol Suppl 1996 Oct;46:73-9; Blackburn WD, Semin Arthritis Rheum 1997 Jun;26(6):788-93)
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
A group of lysosomal proteinases or endopeptidases found in aqueous extracts of a variety of animal tissues. They function optimally within an acidic pH range. The cathepsins occur as a variety of enzyme subtypes including SERINE PROTEASES; ASPARTIC PROTEINASES; and CYSTEINE PROTEASES.
Binary classification measures to assess test results. Sensitivity or recall rate is the proportion of true positives. Specificity is the probability of correctly determining the absence of a condition. (From Last, Dictionary of Epidemiology, 2d ed)
Specific molecular sites on the surface of various cells, including B-lymphocytes and macrophages, that combine with IMMUNOGLOBULIN Gs. Three subclasses exist: Fc gamma RI (the CD64 antigen, a low affinity receptor), Fc gamma RII (the CD32 antigen, a high affinity receptor), and Fc gamma RIII (the CD16 antigen, a low affinity receptor).
An antihelminthic drug that has been tried experimentally in rheumatic disorders where it apparently restores the immune response by increasing macrophage chemotaxis and T-lymphocyte function. Paradoxically, this immune enhancement appears to be beneficial in rheumatoid arthritis where dermatitis, leukopenia, and thrombocytopenia, and nausea and vomiting have been reported as side effects. (From Smith and Reynard, Textbook of Pharmacology, 1991, p435-6)
Serological reactions in which an antiserum against one antigen reacts with a non-identical but closely related antigen.
Immunoglobulin preparations used in intravenous infusion, containing primarily IMMUNOGLOBULIN G. They are used to treat a variety of diseases associated with decreased or abnormal immunoglobulin levels including pediatric AIDS; primary HYPERGAMMAGLOBULINEMIA; SCID; CYTOMEGALOVIRUS infections in transplant recipients, LYMPHOCYTIC LEUKEMIA, CHRONIC; Kawasaki syndrome, infection in neonates, and IDIOPATHIC THROMBOCYTOPENIC PURPURA.
Persistent abnormal dilatation of the bronchi.
The engulfing and degradation of microorganisms; other cells that are dead, dying, or pathogenic; and foreign particles by phagocytic cells (PHAGOCYTES).
Cell-surface glycoprotein beta-chains that are non-covalently linked to specific alpha-chains of the CD11 family of leukocyte-adhesion molecules (RECEPTORS, LEUKOCYTE-ADHESION). A defect in the gene encoding CD18 causes LEUKOCYTE-ADHESION DEFICIENCY SYNDROME.
Elements of limited time intervals, contributing to particular results or situations.
Bleeding or escape of blood from a vessel.
A surgical procedure involving the excision of the COLON and RECTUM and the formation of an ILEOANAL RESERVOIR (pouch). In patients with intestinal diseases, such as ulcerative colitis, this procedure avoids the need for an OSTOMY by allowing for transanal defecation.
The return of a sign, symptom, or disease after a remission.
The presence of organisms, or any foreign material that makes a drug preparation impure.
An immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the Fourth Annual Report on Carcinogens (NTP 85-002, 1985), this substance has been listed as a known carcinogen. (Merck Index, 11th ed)
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
Serum glycoprotein produced by activated MACROPHAGES and other mammalian MONONUCLEAR LEUKOCYTES. It has necrotizing activity against tumor cell lines and increases ability to reject tumor transplants. Also known as TNF-alpha, it is only 30% homologous to TNF-beta (LYMPHOTOXIN), but they share TNF RECEPTORS.
Chronic, non-specific inflammation of the GASTROINTESTINAL TRACT. Etiology may be genetic or environmental. This term includes CROHN DISEASE and ULCERATIVE COLITIS.
Biologically active substances whose activities affect or play a role in the functioning of the immune system.
The pathological process occurring in cells that are dying from irreparable injuries. It is caused by the progressive, uncontrolled action of degradative ENZYMES, leading to MITOCHONDRIAL SWELLING, nuclear flocculation, and cell lysis. It is distinct it from APOPTOSIS, which is a normal, regulated cellular process.
Proteins that are present in blood serum, including SERUM ALBUMIN; BLOOD COAGULATION FACTORS; and many other types of proteins.
Body organ that filters blood for the secretion of URINE and that regulates ion concentrations.
Adherence of cells to surfaces or to other cells.
An acute, febrile, mucocutaneous condition accompanied by swelling of cervical lymph nodes in infants and young children. The principal symptoms are fever, congestion of the ocular conjunctivae, reddening of the lips and oral cavity, protuberance of tongue papillae, and edema or erythema of the extremities.
A chronic transmural inflammation that may involve any part of the DIGESTIVE TRACT from MOUTH to ANUS, mostly found in the ILEUM, the CECUM, and the COLON. In Crohn disease, the inflammation, extending through the intestinal wall from the MUCOSA to the serosa, is characteristically asymmetric and segmental. Epithelioid GRANULOMAS may be seen in some patients.
Chronic inflammatory disease of the BILIARY TRACT. It is characterized by fibrosis and hardening of the intrahepatic and extrahepatic biliary ductal systems leading to bile duct strictures, CHOLESTASIS, and eventual BILIARY CIRRHOSIS.
Highly reactive compounds produced when oxygen is reduced by a single electron. In biological systems, they may be generated during the normal catalytic function of a number of enzymes and during the oxidation of hemoglobin to METHEMOGLOBIN. In living organisms, SUPEROXIDE DISMUTASE protects the cell from the deleterious effects of superoxides.
A group of CORTICOSTEROIDS that affect carbohydrate metabolism (GLUCONEOGENESIS, liver glycogen deposition, elevation of BLOOD SUGAR), inhibit ADRENOCORTICOTROPIC HORMONE secretion, and possess pronounced anti-inflammatory activity. They also play a role in fat and protein metabolism, maintenance of arterial blood pressure, alteration of the connective tissue response to injury, reduction in the number of circulating lymphocytes, and functioning of the central nervous system.
Leukocytes with abundant granules in the cytoplasm. They are divided into three groups according to the staining properties of the granules: neutrophilic, eosinophilic, and basophilic. Mature granulocytes are the NEUTROPHILS; EOSINOPHILS; and BASOPHILS.
Univalent antigen-binding fragments composed of one entire IMMUNOGLOBULIN LIGHT CHAIN and the amino terminal end of one of the IMMUNOGLOBULIN HEAVY CHAINS from the hinge region, linked to each other by disulfide bonds. Fab contains the IMMUNOGLOBULIN VARIABLE REGIONS, which are part of the antigen-binding site, and the first IMMUNOGLOBULIN CONSTANT REGIONS. This fragment can be obtained by digestion of immunoglobulins with the proteolytic enzyme PAPAIN.
The technique of using FIXATIVES in the preparation of cytologic, histologic, or pathologic specimens for the purpose of maintaining the existing form and structure of all the constituent elements.
A chronic self-perpetuating hepatocellular INFLAMMATION of unknown cause, usually with HYPERGAMMAGLOBULINEMIA and serum AUTOANTIBODIES.
A member of the CXC chemokine family that plays a role in the regulation of the acute inflammatory response. It is secreted by variety of cell types and induces CHEMOTAXIS of NEUTROPHILS and other inflammatory cells.
The part of a cell that contains the CYTOSOL and small structures excluding the CELL NUCLEUS; MITOCHONDRIA; and large VACUOLES. (Glick, Glossary of Biochemistry and Molecular Biology, 1990)
Small cationic peptides that are an important component, in most species, of early innate and induced defenses against invading microbes. In animals they are found on mucosal surfaces, within phagocytic granules, and on the surface of the body. They are also found in insects and plants. Among others, this group includes the DEFENSINS, protegrins, tachyplesins, and thionins. They displace DIVALENT CATIONS from phosphate groups of MEMBRANE LIPIDS leading to disruption of the membrane.
Chemical substances that attract or repel cells. The concept denotes especially those factors released as a result of tissue injury, microbial invasion, or immunologic activity, that attract LEUKOCYTES; MACROPHAGES; or other cells to the site of infection or insult.
A large increase in oxygen uptake by neutrophils and most types of tissue macrophages through activation of an NADPH-cytochrome b-dependent oxidase that reduces oxygen to a superoxide. Individuals with an inherited defect in which the oxidase that reduces oxygen to superoxide is decreased or absent (GRANULOMATOUS DISEASE, CHRONIC) often die as a result of recurrent bacterial infections.
The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.
Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.
The processes triggered by interactions of ANTIBODIES with their ANTIGENS.
Conditions in which the KIDNEYS perform below the normal level in the ability to remove wastes, concentrate URINE, and maintain ELECTROLYTE BALANCE; BLOOD PRESSURE; and CALCIUM metabolism. Renal insufficiency can be classified by the degree of kidney damage (as measured by the level of PROTEINURIA) and reduction in GLOMERULAR FILTRATION RATE.
An adhesion-promoting leukocyte surface membrane heterodimer. The alpha subunit consists of the CD11b ANTIGEN and the beta subunit the CD18 ANTIGEN. The antigen, which is an integrin, functions both as a receptor for complement 3 and in cell-cell and cell-substrate adhesive interactions.
Proteins prepared by recombinant DNA technology.
Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake.
The measurement of infection-blocking titer of ANTISERA by testing a series of dilutions for a given virus-antiserum interaction end-point, which is generally the dilution at which tissue cultures inoculated with the serum-virus mixtures demonstrate cytopathology (CPE) or the dilution at which 50% of test animals injected with serum-virus mixtures show infectivity (ID50) or die (LD50).
The number of WHITE BLOOD CELLS per unit volume in venous BLOOD. A differential leukocyte count measures the relative numbers of the different types of white cells.
Antibodies that inhibit the reaction between ANTIGEN and other antibodies or sensitized T-LYMPHOCYTES (e.g., antibodies of the IMMUNOGLOBULIN G class that compete with IGE antibodies for antigen, thereby blocking an allergic response). Blocking antibodies that bind tumors and prevent destruction of tumor cells by CYTOTOXIC T-LYMPHOCYTES have also been called enhancing antibodies. (Rosen et al., Dictionary of Immunology, 1989)
Therapeutic act or process that initiates a response to a complete or partial remission level.
Rare chronic inflammatory disease involving the small blood vessels. It is of unknown etiology and characterized by mucocutaneous ulceration in the mouth and genital region and uveitis with hypopyon. The neuro-ocular form may cause blindness and death. SYNOVITIS; THROMBOPHLEBITIS; gastrointestinal ulcerations; RETINAL VASCULITIS; and OPTIC ATROPHY may occur as well.
Antibodies, often monoclonal, in which the two antigen-binding sites are specific for separate ANTIGENIC DETERMINANTS. They are artificial antibodies produced by chemical crosslinking, fusion of HYBRIDOMA cells, or by molecular genetic techniques. They function as the main mediators of targeted cellular cytotoxicity and have been shown to be efficient in the targeting of drugs, toxins, radiolabeled haptens, and effector cells to diseased tissue, primarily tumors.
Abnormal immunoglobulins, especially IGG or IGM, that precipitate spontaneously when SERUM is cooled below 37 degrees Celsius. It is characteristic of CRYOGLOBULINEMIA.
Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons.
Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder.
Studies which start with the identification of persons with a disease of interest and a control (comparison, referent) group without the disease. The relationship of an attribute to the disease is examined by comparing diseased and non-diseased persons with regard to the frequency or levels of the attribute in each group.
A characteristic symptom complex.
Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease.
A highly reactive aldehyde gas formed by oxidation or incomplete combustion of hydrocarbons. In solution, it has a wide range of uses: in the manufacture of resins and textiles, as a disinfectant, and as a laboratory fixative or preservative. Formaldehyde solution (formalin) is considered a hazardous compound, and its vapor toxic. (From Reynolds, Martindale The Extra Pharmacopoeia, 30th ed, p717)
A protease of broad specificity, obtained from dried pancreas. Molecular weight is approximately 25,000. The enzyme breaks down elastin, the specific protein of elastic fibers, and digests other proteins such as fibrin, hemoglobin, and albumin. EC 3.4.21.36.
Therapy with two or more separate preparations given for a combined effect.
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
A cluster of convoluted capillaries beginning at each nephric tubule in the kidney and held together by connective tissue.
A form of antibodies consisting only of the variable regions of the heavy and light chains (FV FRAGMENTS), connected by a small linker peptide. They are less immunogenic than complete immunoglobulin and thus have potential therapeutic use.
Pathological processes of the KIDNEY or its component tissues.
Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.
Serum that contains antibodies. It is obtained from an animal that has been immunized either by ANTIGEN injection or infection with microorganisms containing the antigen.
Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood.
Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function.
Immunologic techniques based on the use of: (1) enzyme-antibody conjugates; (2) enzyme-antigen conjugates; (3) antienzyme antibody followed by its homologous enzyme; or (4) enzyme-antienzyme complexes. These are used histologically for visualizing or labeling tissue specimens.
Differentiation antigens residing on mammalian leukocytes. CD stands for cluster of differentiation, which refers to groups of monoclonal antibodies that show similar reactivity with certain subpopulations of antigens of a particular lineage or differentiation stage. The subpopulations of antigens are also known by the same CD designation.
Small polyhedral outpouchings along the walls of the alveolar sacs, alveolar ducts and terminal bronchioles through the walls of which gas exchange between alveolar air and pulmonary capillary blood takes place.
The major metabolite in neutrophil polymorphonuclear leukocytes. It stimulates polymorphonuclear cell function (degranulation, formation of oxygen-centered free radicals, arachidonic acid release, and metabolism). (From Dictionary of Prostaglandins and Related Compounds, 1990)
Purplish or brownish red discoloration, easily visible through the epidermis, caused by hemorrhage into the tissues. When the size of the discolorization is >2-3 cm it is generally called Ecchymoses (ECCHYMOSIS).
A systemic autoimmune disorder that typically affects medium and large ARTERIES, usually leading to occlusive granulomatous vasculitis with transmural infiltrate containing multinucleated GIANT CELLS. The TEMPORAL ARTERY is commonly involved. This disorder appears primarily in people over the age of 50. Symptoms include FEVER; FATIGUE; HEADACHE; visual impairment; pain in the jaw and tongue; and aggravation of pain by cold temperatures. (From Adams et al., Principles of Neurology, 6th ed)
Disorders related or resulting from use of cocaine.
Established cell cultures that have the potential to propagate indefinitely.
Antibodies elicited in a different species from which the antigen originated. These antibodies are directed against a wide variety of interspecies-specific antigens, the best known of which are Forssman, Hanganutziu-Deicher (H-D), and Paul-Bunnell (P-B). Incidence of antibodies to these antigens--i.e., the phenomenon of heterophile antibody response--is useful in the serodiagnosis, pathogenesis, and prognosis of infection and latent infectious states as well as in cancer classification.
Antibodies that can catalyze a wide variety of chemical reactions. They are characterized by high substrate specificity and share many mechanistic features with enzymes.
Methods used for studying the interactions of antibodies with specific regions of protein antigens. Important applications of epitope mapping are found within the area of immunochemistry.
Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group.
Antibodies from non-human species whose protein sequences have been modified to make them nearly identical with human antibodies. If the constant region and part of the variable region are replaced, they are called humanized. If only the constant region is modified they are called chimeric. INN names for humanized antibodies end in -zumab.
Lymphocytes responsible for cell-mediated immunity. Two types have been identified - cytotoxic (T-LYMPHOCYTES, CYTOTOXIC) and helper T-lymphocytes (T-LYMPHOCYTES, HELPER-INDUCER). They are formed when lymphocytes circulate through the THYMUS GLAND and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen.
White blood cells. These include granular leukocytes (BASOPHILS; EOSINOPHILS; and NEUTROPHILS) as well as non-granular leukocytes (LYMPHOCYTES and MONOCYTES).
Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).
The rate dynamics in chemical or physical systems.
Lipid-containing polysaccharides which are endotoxins and important group-specific antigens. They are often derived from the cell wall of gram-negative bacteria and induce immunoglobulin secretion. The lipopolysaccharide molecule consists of three parts: LIPID A, core polysaccharide, and O-specific chains (O ANTIGENS). When derived from Escherichia coli, lipopolysaccharides serve as polyclonal B-cell mitogens commonly used in laboratory immunology. (From Dorland, 28th ed)
Autoantibodies directed against phospholipids. These antibodies are characteristically found in patients with systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC;), ANTIPHOSPHOLIPID SYNDROME; related autoimmune diseases, some non-autoimmune diseases, and also in healthy individuals.
Antigens on surfaces of cells, including infectious or foreign cells or viruses. They are usually protein-containing groups on cell membranes or walls and may be isolated.
In screening and diagnostic tests, the probability that a person with a positive test is a true positive (i.e., has the disease), is referred to as the predictive value of a positive test; whereas, the predictive value of a negative test is the probability that the person with a negative test does not have the disease. Predictive value is related to the sensitivity and specificity of the test.
A chronic inflammatory process that affects the AORTA and its primary branches, such as the brachiocephalic artery (BRACHIOCEPHALIC TRUNK) and CAROTID ARTERIES. It results in progressive arterial stenosis, occlusion, and aneurysm formation. The pulse in the arm is hard to detect. Patients with aortitis syndrome often exhibit retinopathy.
The movement of cells from one location to another. Distinguish from CYTOKINESIS which is the process of dividing the CYTOPLASM of a cell.
A dermal inflammatory reaction produced under conditions of antibody excess, when a second injection of antigen produces intravascular antigen-antibody complexes which bind complement, causing cell clumping, endothelial damage, and vascular necrosis.
Substances that are recognized by the immune system and induce an immune reaction.
Lymphoid cells concerned with humoral immunity. They are short-lived cells resembling bursa-derived lymphocytes of birds in their production of immunoglobulin upon appropriate stimulation.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.
The worsening of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.
The minor fragment formed when C5 convertase cleaves C5 into C5a and COMPLEMENT C5B. C5a is a 74-amino-acid glycopeptide with a carboxy-terminal ARGININE that is crucial for its spasmogenic activity. Of all the complement-derived anaphylatoxins, C5a is the most potent in mediating immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE), smooth MUSCLE CONTRACTION; HISTAMINE RELEASE; and migration of LEUKOCYTES to site of INFLAMMATION.
Condensed areas of cellular material that may be bounded by a membrane.
The relatively long-lived phagocytic cell of mammalian tissues that are derived from blood MONOCYTES. Main types are PERITONEAL MACROPHAGES; ALVEOLAR MACROPHAGES; HISTIOCYTES; KUPFFER CELLS of the liver; and OSTEOCLASTS. They may further differentiate within chronic inflammatory lesions to EPITHELIOID CELLS or may fuse to form FOREIGN BODY GIANT CELLS or LANGHANS GIANT CELLS. (from The Dictionary of Cell Biology, Lackie and Dow, 3rd ed.)
The sum of the weight of all the atoms in a molecule.
Procedure whereby plasma is separated and extracted from anticoagulated whole blood and the red cells retransfused to the donor. Plasmapheresis is also employed for therapeutic use.
Cells artificially created by fusion of activated lymphocytes with neoplastic cells. The resulting hybrid cells are cloned and produce pure MONOCLONAL ANTIBODIES or T-cell products, identical to those produced by the immunologically competent parent cell.
A CXC chemokine with specificity for CXCR2 RECEPTORS. It has growth factor activities and is implicated as a oncogenic factor in several tumor types.
Substances that reduce or suppress INFLAMMATION.
Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.
Deliberate stimulation of the host's immune response. ACTIVE IMMUNIZATION involves administration of ANTIGENS or IMMUNOLOGIC ADJUVANTS. PASSIVE IMMUNIZATION involves administration of IMMUNE SERA or LYMPHOCYTES or their extracts (e.g., transfer factor, immune RNA) or transplantation of immunocompetent cell producing tissue (thymus or bone marrow).
High-affinity G-protein-coupled receptors for INTERLEUKIN-8 present on NEUTROPHILS; MONOCYTES; and T-LYMPHOCYTES. These receptors also bind several other CXC CHEMOKINES.
Surface ligands, usually glycoproteins, that mediate cell-to-cell adhesion. Their functions include the assembly and interconnection of various vertebrate systems, as well as maintenance of tissue integration, wound healing, morphogenic movements, cellular migrations, and metastasis.
Large, phagocytic mononuclear leukocytes produced in the vertebrate BONE MARROW and released into the BLOOD; contain a large, oval or somewhat indented nucleus surrounded by voluminous cytoplasm and numerous organelles.
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
Non-antibody proteins secreted by inflammatory leukocytes and some non-leukocytic cells, that act as intercellular mediators. They differ from classical hormones in that they are produced by a number of tissue or cell types rather than by specialized glands. They generally act locally in a paracrine or autocrine rather than endocrine manner.
Antibodies found in adult RHEUMATOID ARTHRITIS patients that are directed against GAMMA-CHAIN IMMUNOGLOBULINS.
Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.
The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
Tomography using x-ray transmission and a computer algorithm to reconstruct the image.
Substances elaborated by bacteria that have antigenic activity.
Glycoproteins found on the membrane or surface of cells.
The process of losing secretory granules (SECRETORY VESICLES). This occurs, for example, in mast cells, basophils, neutrophils, eosinophils, and platelets when secretory products are released from the granules by EXOCYTOSIS.
A flavoprotein enzyme that catalyzes the univalent reduction of OXYGEN using NADPH as an electron donor to create SUPEROXIDE ANION. The enzyme is dependent on a variety of CYTOCHROMES. Defects in the production of superoxide ions by enzymes such as NADPH oxidase result in GRANULOMATOUS DISEASE, CHRONIC.

Predictors of treatment resistance and relapse in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis: comparison of two independent cohorts. (1/99)

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Chimeric IgG4 PR3-ANCA induces selective inflammatory responses from neutrophils through engagement of Fcgamma receptors. (2/99)

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CT60 and +49 polymorphisms of CTLA 4 are associated with ANCA-positive small vessel vasculitis. (3/99)

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Increased incidence of cardiovascular events in patients with antineutrophil cytoplasmic antibody-associated vasculitides: a matched-pair cohort study. (4/99)

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Small vessel vasculitis. (5/99)

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Spontaneous resolution of delayed onset large subclavian artery pseudoaneurysm. (6/99)

A 70-year-old woman with a history of end-stage renal disease secondary to anti-neutrophil cytoplasmic autoantibody-associated vasculitis had been receiving hemodialysis for 5 years. The patient underwent attempted right internal jugular vein cannulation for temporary hemodialysis catheter placement. Pulsating mass developed in the neck and angiography revealed a subclavian artery pseudoaneurysm 4 days later. The pseudoaneurysm disappeared spontaneously during the interval between the diagnosis and the planned surgical procedure. Such delayed onset and spontaneous resolution of subclavian artery pseudoaneurysm is uncommon. Close observation may be optimal if delayed onset of pseudoaneurysm occurs after small needle puncture with cessation of antiplatelet/anticoagulant administration.  (+info)

Confirmation of the genetic association of CTLA4 and PTPN22 with ANCA-associated vasculitis. (7/99)

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Prediction of ESRD in pauci-immune necrotizing glomerulonephritis: quantitative histomorphometric assessment and serum creatinine. (8/99)

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TY - JOUR. T1 - Association between age at disease onset of anti-neutrophil cytoplasmic antibody-associated vasculitis and clinical presentation and short-term outcomes. AU - Monti, Sara. AU - Craven, Anthea. AU - Klersy, Catherine. AU - Montecucco, Carlomaurizio. AU - Caporali, Roberto. AU - Watts, Richard. AU - Merkel, Peter A.. AU - Luqmani, Raashid. AU - Achilleos, Katerina. AU - Adler, Matthew. AU - Alba, Marco A.. AU - Albert, Daniel A.. AU - Alibaz-Oner, Fatma. AU - Allcoat, Paul. AU - Amano, Koichi. AU - Amarasuriya, Manishka. AU - Amudala, Naomi A.. AU - Andrews, Jacqueline. AU - Archer, Amy M.. AU - Arimura, Yoshihiro. AU - Atukorala, Inoshi. AU - Azevedo, Elsa. AU - Bajad, Shruti. AU - Baldwin, Corisande. AU - Barra, Lillian J.. AU - Baslund, Bo. AU - Basu, Neil. AU - Baykal, Mahire. AU - Berger, Christoph. AU - Berglin, Ewa. AU - Besada, Emilio. AU - Bhardwaj, Mamta. AU - Bischof, Antje. AU - Blockmans, Daniel. AU - Blood, Janet. AU - Draibe, Juliana Bordignon. AU - Brand, ...
Rituximab for refractory granulomatous eye disease Elyse E Lower1,2 Robert P Baughman,1 Adam H Kaufman31Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA; 2Oncology Hematology Care, Cincinnati, OH, USA; 3Department of Ophthalmology, University of Cincinnati, Cincinnati, OH, USAObjective: To determine the effectiveness of rituximab therapy for patients with granulomatous disease of the eye.Methods: Retrospective review was undertaken of cases seen at a single institution for ocular antineutrophil cytoplasmic antibody-associated vasculitis or sarcoidosis with persistent ocular disease despite systemic therapy. All patients were treated with rituximab and followed for at least 6 months.Results: Nine patients were identified (five with antineutrophil cytoplasmic antibody-associated vasculitis, four with sarcoidosis), and all were treated for at least 6 months. Eight experienced improvement of eye disease and were able to reduce prednisone and other drug therapies. One
Evidence-based recommendations on rituximab (MabThera) with glucocorticoids for treating anti-neutrophil cytoplasmic antibody-associated vasculitis
Evidence-based recommendations on rituximab (MabThera) with glucocorticoids for treating anti-neutrophil cytoplasmic antibody-associated vasculitis
Background Current recommendations for ANCA-associated vasculitis (AAV) support its management within a dedicated clinical service. Therapies for AAV are imperfect with many patients failing to...
The presence of ENT involvement in AAV patients is associated with prognostically favourable renal biopsy findings and better renal function. These results indicate that there may be different phenotypes of AAV defined by ENT involvement.
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If patients could recognise themselves, or anyone else could recognise a patient from your description, please obtain the patients written consent to publication and send them to the editorial office before submitting your response [Patient consent forms] ...
Wet-lab validated real-time PCR primer assays for your biological pathway of interest. Select your gene target of interest using an interactive pathway map, and select your plate.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a heterogeneous group of diseases corresponding to necrotising inflammation of small vessels with a wide range of clinical presentations. At least two of the diseases are believed to exhibit a common ground of pathophysiological mechanisms. These are granulomatosis with polyangiitis (GPA, formerly known as Wegeners granulomatosis) and microscopic polyangiitis (MPA). ANCA directed against proteinase 3 (PR3) are preferentially associated with GPA, and anti-myeloperoxidase (MPO) ANCA are associated mainly with MPA and eosinophilic GPA (formerly known as Churg-Strauss syndrome). Anti-MPO and anti-PR3 antibodies can activate neutrophils in vitro. In vivo data are available for humans and mice on the pathogenicity of anti-MPO but it is more controversial for PR3-ANCA. A recent genome-wide association study of patients with ANCA-associated vasculitides confirmed the genetic contribution to the pathogenesis of these conditions, with ...
Clinical trial for Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis | Microscopic Polyangiitis | Wegeners Granulomatosis , Low-dose Glucocorticoid Vasculitis Induction Study
Objective. Treatment resistance and relapse in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are major challenges for physicians. The aim of this study was to assess the risk factors for treatment resistance and relapse in a single-center cohort of Chinese patients with AAV.. Methods. Four hundred thirty-nine consecutive patients with AAV were recruited for inclusion in this study. The value of various clinical and pathologic parameters for the prediction of treatment resistance and relapse was analyzed.. Results. Treatment resistance occurred in 47 (10.7%) of 439 patients and was independently associated with a higher serum creatinine level (odds ratio [OR] 1.087, 95% confidence interval [95% CI] 1.001-1.180, P = 0.047), a higher erythrocyte sedimentation rate (OR 1.009, 95% CI 1.001-1.018, P = 0.025), therapy with corticosteroids plus cyclophosphamide (OR 0.115, 95% CI 0.051-0.256, P = 0.000), and the presence of muscle pain (OR 0.249, 95% CI 0.083-0.747, P = 0.013). ...
The next 3 papers were recently published in NDT. M Huber et al first discuss Should belatacept be the centrepiece of renal transplantation?. Then J Perl et al report on Association between changes in quality of life and mortality in hemodialysis patients: results from the DOPPS. And JF Sanders et al report on Extended versus standard azathioprine maintenance therapy in newly diagnosed proteinase-3 anti-neutrophil cytoplasmic antibody-associated vasculitis patients who remain cytoplasmic anti-neutrophil cytoplasmic antibody-positive after induction of remission: a randomized clinical trial ...
TY - JOUR. T1 - Epitope specificity determines pathogenicity and detectability in anca-associated vasculitis. AU - Roth, Aleeza J.. AU - Ooi, Joshua D.. AU - Hess, Jacob J.. AU - Van Timmeren, Mirjan M.. AU - Berg, Elisabeth A.. AU - Poulton, Caroline E.. AU - McGregor, Julie Anne. AU - Burkart, Madelyn. AU - Hogan, Susan L.. AU - Hu, Yichun. AU - Winnik, Witold. AU - Nachman, Patrick H.. AU - Stegeman, Coen A.. AU - Niles, John. AU - Heeringa, Peter. AU - Kitching, A. Richard. AU - Holdsworth, Stephen. AU - Jennette, J. Charles. AU - Preston, Gloria A.. AU - Falk, Ronald J.. PY - 2013/4/1. Y1 - 2013/4/1. N2 - Anti-neutrophil cytoplasmic antibody-associated (ANCA-associated) small vessel necrotizing vasculitis is caused by immune-mediated inflammation of the vessel wall and is diagnosed in some cases by the presence of myeloperoxidase-specific antibodies (MPO-ANCA). This multicenter study sought to determine whether differences in ANCA epitope specificity explain why, in some cases, conventional ...
Objectives: Glucocorticoids (GCs) are a mainstay of treatment for patients with ANCA-associated vasculitis (AAV) but are associated with significant adverse effects. Effective remission induction in severe AAV using extremely limited GC exposure has not been attempted. We tested an early rapid GC withdrawal induction regimen for patients with severe AAV. Methods: Patients with active MPO- or PR3-ANCA vasculitis or ANCA-negative pauci-immune glomerulonephritis were included. Induction treatment consisted of two doses of rituximab, 3 months of low-dose CYC and a short course of oral GC (for between 1 and 2 weeks). Clinical, biochemical and immunological outcomes as well as adverse events were recorded. Results: A total of 49 patients were included, with at least 12 months of follow-up in 46. All patients achieved remission, with decreases observed in creatinine, proteinuria, CRP, ANCA level and BVAS. Three patients requiring dialysis at presentation became dialysis independent. Two patients ...
Plasminogen antibody (Biotin) (plasminogen) for ELISA, WB. Anti-Plasminogen pAb (GTX79699) is tested in Human samples. 100% Ab-Assurance.
Introduction: Evidence from a number of open-label, uncontrolled studies has suggested that rituximab may benefit patients with autoimmune diseases who are refractory to standard-of-care. The objective of this study was to evaluate the safety and clinical outcomes of rituximab in several standard-of-care-refractory autoimmune diseases (within rheumatology, nephrology, dermatology and neurology) other than rheumatoid arthritis or non-Hodgkins lymphoma in a real-life clinical setting. Methods: Patients who received rituximab having shown an inadequate response to standard-of-care had their safety and clinical outcomes data retrospectively analysed as part of the German Registry of Autoimmune Diseases. The main outcome measures were safety and clinical response, as judged at the discretion of the investigators. Results: A total of 370 patients (299 patient-years) with various autoimmune diseases (23.0% with systemic lupus erythematosus, 15.7% antineutrophil cytoplasmic antibody-associated ...
Introduction: Evidence from a number of open-label, uncontrolled studies has suggested that rituximab may benefit patients with autoimmune diseases who are refractory to standard-of-care. The objective of this study was to evaluate the safety and clinical outcomes of rituximab in several standard-of-care-refractory autoimmune diseases (within rheumatology, nephrology, dermatology and neurology) other than rheumatoid arthritis or non-Hodgkins lymphoma in a real-life clinical setting. Methods: Patients who received rituximab having shown an inadequate response to standard-of-care had their safety and clinical outcomes data retrospectively analysed as part of the German Registry of Autoimmune Diseases. The main outcome measures were safety and clinical response, as judged at the discretion of the investigators. Results: A total of 370 patients (299 patient-years) with various autoimmune diseases (23.0% with systemic lupus erythematosus, 15.7% antineutrophil cytoplasmic antibody-associated ...
Purpose: Combined use of cyclophosphamide (CYC) and glucocorticoids (GCS) has been the standard of care for remission induction for ANCA-associated vasculitis (AAV) for decades. Uncontrolled studies suggest rituximab (RTX) may be effective for AAV, and its use may avoid some of the toxicities associated with CYC therapy. This trial compares the efficacy of RTX to that of CYC for AAV.. Method: A multicenter, randomized, double-blind, placebo-controlled trial was conducted to determine if treatment with RTX (375 mg/m2 i.v. weekly x 4) was not inferior to CYC (2 mg/kg/d p.o.) for inducing remission in severe AAV. Once remission was achieved, CYC was replaced by azathioprine between months 3-6. All patients received the same GCS treatment protocol: 1-3 g i.v. methylprednisolone followed by prednisone 1 mg/kg/d p.o. reduced to 40 mg/d by month 1, and then tapered and discontinued completely by month 6. The primary endpoint was disease remission in the absence of prednisone therapy at month 6. ...
A study suggests that platelets are key contributors to ANCA-associated vasculitis via activation of the alternative complement pathway.
--Oral presentation at the American Society of Nephrology Kidney Week 2016 to highlight Phase II AAV CLEAR trial results--. --Oral presentation at the American College of Rheumatology 2016 Annual Meeting to highlight Phase II AAV CLASSIC trial results--. MOUNTAIN VIEW, Calif., Nov. 07, 2016-- ChemoCentryx, Inc.,, today announced oral presentations at two upcoming...
MOUNTAIN VIEW, Calif., June 2, 2014 (GLOBE NEWSWIRE) -- ChemoCentryx, Inc., (Nasdaq:CCXI) reported today additional Phase II data related to CCX168, an orally administered inhibitor that targets the receptor for the complement protein known as C5a (C5aR). Data were presented in an oral presentation at the 51
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Read about a study that found patients with ANCA-associated vasculitis carry a threefold and eightfold higher risk for cardiovascular disease and cerebrovascular accidents, respectively, compared to the general population.
国内在庫あります!FITC標識済みシープ・ポリクローナル抗体 ab27617 交差種: Hu 適用: ICC/IF…Plasminogen抗体一覧 一次抗体にFITCを直接標識し、操作時間の短縮と低いバックグラウンドを実現。
Randomized clinical trials in ANCA-associated vasculitis: a systematic analysis of the WHO - International Clinical Trials Registry Platform. Crossref DOI link: https://doi.org/10.1186/S13023-020-01408-6 Published: 2020-12. Update policy: https://doi.org/10.1007/SPRINGER_CROSSMARK_POLICY. ...
The abstract of this paper is available from the Lancet. However, you must register at the Lancet site first as a non-subscriber (you can get there via the journals page of HDCN ...
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Background Secondary pulmonary hemorrhage increases the risk of mortality in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV); plasma exchange therapy may improve outcomes in these patients. We conducted a retrospective cohort study to investigate the effect of plasma exchange therapy on short-term prognoses in patients with pulmonary hemorrhage secondary to AAV. Methods This study utilized the Diagnosis Procedure Combination database, which is a nationwide inpatient database in Japan. We checked the abstract data and medical actions and identified the patients with pulmonary hemorrhage secondary to AAV who required proactive treatment between 2009 and 2014. To compare the in-hospital mortality, we performed propensity score matching between the plasma exchange and non-plasma exchange groups at a ratio of 1:1. Results Of the 52,932 patients with AAV, 940 developed pulmonary hemorrhage as a complication. A total of 249 patients from 194 hospitals were eligible for the
Antineutrophil cytoplasmic antibodies (ANCA) and Anti-glomerular basement membrane (GBM) antibodies often induce rapidly progressive glomerulonephritis (RPGN). Some reports have demonstrated RPGN with the sequential appearance of ANCA then anti-GBM antibodies, suggesting that ANCA may induce the development of anti-GBM antibodies. Whereas, many reports have shown that the development of ANCA is associated with various infectious diseases, such as non-tuberculous mycobacterial infection. A 65-year-old woman with pulmonary non-tuberculous mycobacterial (NTM) infection was monitored without treatment. One year later, serum myeloperoxidase (MPO)- ANCA were elevated (14.1 U/mL (normal value | 3.0 U/ml)). A high fever and RPGN appeared 1 year later, and serum MPO-ANCAs were 94.1 U/mL. Anti-GBM antibodies were also detected. A renal biopsy revealed crescentic glomerulonephritis with linear deposits of IgG and C3c along the GBM and interstitial inflammation with endarteritis of arterioles. The diagnosis was
The etiology of anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitides (AAV) is unknown, but the association between infections and autoimmunity has been studied extensively. In 2004, a novel theory was proposed that could link infection and autoimmunity. This theory of autoantigen complementarity was based on the serendipitous finding of antibodies against complementary-PR3 (cPR3) in patients with PR3-ANCA-associated vasculitis. cPR3 demonstrated homology to several bacterial proteins, and it was hypothesized that PR3-ANCA develop in response to anti-cPR3 antibodies, as a consequence of the anti-idiotypic network. These data have not been confirmed in other patient cohorts. We investigated the presence of anti-cPR3 antibodies in a Dutch cohort of PR3-ANCA-associated vasculitis patients. Anti-cPR3 reactivity was determined in serum using ELISA. Two separate batches of cPR3 were used to determine reactivity in two separate cohorts of PR3-ANCA-associated vasculitis patients. We ...
Lupus anticoagulant has been described in association with many autoimmune disorders. Here we describe its occurrence in a patient with ANCA-associated microscopic polyarteritis with medium vessel involvement. A 62-year-old man presented with mononeuritis multiplex and abdominal pain and was demonst …
The primary safety objective of this study is to evaluate the safety and tolerability of CCX168 in subjects with AAV on background cyclophosphamide or rituximab treatment.. The primary efficacy objective is to evaluate the efficacy of CCX168 based on the Birmingham Vasculitis Activity Score (BVAS) version 3.. The secondary objectives of this study include assessment of the feasibility of reducing or eliminating the use of corticosteroids in the treatment of subjects with ANCA-associated vasculitis without the need for rescue corticosteroid measures and the effect of CCX168 on several disease parameters. ...
In this study, we investigated the mechanism of platelet activation in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), as well as the activation of the alternative complement pathway by platelets in AAV. CD62P and platelet-leukocyte aggregates in AAV patients were tested by flow cytometry. Platelets were stimulated by plasma from active AAV patients. The effect of the thrombin-protease-activated receptors (PARs) pathway was evaluated by blocking thrombin or PAR1 antagonists. After platelets were activated by plasma from AAV patients, Ca/Mg-Tyrodes buffer and Mg-EGTA buffer were used to measure complement activation in liquid phase and on the surface of platelets. The levels of CD62P-expressing platelets and platelet-leukocyte aggregates were significantly higher in active AAV patients than those in remission and normal controls. Platelets were activated by plasma from active AAV patients (percentage of CD62P-expressing platelets, 97.7 ± 3% vs. 1 ± 0.2%, p | 0
Most patients with ANCA-associated vasculitis will not present with visual or other overt signs of renal involvement: macroscopic haematuria is usually absent as one of the presenting symptoms and proteinuria is frequently mild, not leading to the clinical signs of nephrotic syndrome. This is also nicely illustrated by the paper by Houben as only 21 of the patients from their cohort were diagnosed by the renal department. This means that patients present with combinations of other signs and symptoms and are referred to other disciplines. Renal involvement, therefore, has to be actively investigated once the diagnosis of ANCA-associated vasculitis is suspected. This means that in every patient in whom the diagnosis of ANCA-associated vasculitis is seriously considered, assessment of renal function (serum creatinine, estimated glomerular filtration rate (eGFR), 24-hour urine creatinine clearance) and urinalysis (erythrocyturia and if present urinary microscopy for glomerular erythrocyturia and/or ...
Objectives: There are few data on clinical profiles of ANCA-associated vasculitis (AAV) in different ethnic populations. The aim of this study was to examine the differences in the ANCA type and clinical features of AAV between populations using the Diagnostic and Classification Criteria in Vasculitis Study (DCVAS) dataset. Methods: The DCVAS is an international, multicentre, observational study recruiting in 133 sites. Eight ethnic categories were analysed: Northern European, Caucasian American, Southern European, Middle Eastern/Turkish, Chinese, Japanese, Indian subcontinent and other. ANCA type was categorized as myeloperoxidase (MPO), PR3 and ANCA negative. Organ system involvement was recorded using a standard dataset. Differences were analysed by chi-squared tests using a Bonferroni correction and logistic regression (adjusting for age and sex). Northern European was the reference population. Results: Data from 1217 patients with AAV were available and the 967 (79.5%) patients recruited by
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The main result of our analysis is that in AAV patients with predominant renal and pulmonary involvement, comorbidity score independently predicted short term survival. It also proved to be a predictor of infectious mortality. On protocolized immunosuppressive therapy, patients, who had high BVAS at baseline, had significantly poorer short term survival and more frequent relapses than subjects with lower than median score. When analyzing early and late mortality separately, BVAS did not predict outcome in univariable analysis.. BVAS, originally designed to standardize disease assessment in AAV, shows good correlation with clinical activity of the disease [8]. Flossmann et al. documented, that BVAS was a significant predictor of mortality by analyzing the data of patients recruited for randomized controlled trials. Patients in that study were somewhat different from the ones enrolled in ours, since the median BVAS was lower, renal function was less severely compromised, and subjects with ...
The treatment strategy for MPA follows that of GPA, as most of the data regarding treatment comes from trials that have included patients with both diseases (under the term ANCA associated vasculitis). Treatment should be urgently instituted after confirming the diagnosis. In patients with severe organ-threatening or life-threatening manifestations, high dose glucocorticoids may be used even while diagnostic confirmation is in progress. Treatment of MPA involves two-stages: induction of remission and maintenance of remission. Induction therapy includes high dose glucocorticoids (typically prednisone 1 mg/kg/day with or without preceding intravenous methylprednisolone 1000 mg for 3 doses) along with methotrexate (MTX) or rituximab (RTX) for non-severe disease and cyclophosphamide (CYC) or RTX for severe disease should be used. Untreated severe MPA has high mortality. Glucocorticoids form the cornerstone of treatment. The choice of the second agent is determined by disease severity, which can be ...
A monoclonal antibody may pose less of a malignancy risk than cyclophosphamide in certain patients with vasculitis, researchers report.
Antibody-mediated and paraneoplastic autoimmune encephalitides (AE) present with a broad spectrum of clinical symptoms. They often lead to progressing inflammatory changes of the central nervous system with subacute onset and can cause persistent brain damage. Thus, to promptly start the appropriate and AE-specific therapy, recognition of symptoms, initiation of relevant antibody diagnostics and confirmation of the clinical diagnosis are crucial, in particular as the diseases are relatively rare. This standard operating procedure (SOP) should draw attention to the clinical presentation of AE, support the diagnostic approach to patients with suspected AE and guide through the necessary steps including therapeutic decisions, tumour screening and exclusion of differential diagnoses. Based on existing diagnostic algorithms, treatment recommendations and personal experiences, this SOP gives an overview of clinical presentation, diagnostic procedures and therapy in AE. Additional information is provided
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SAN DIEGO -- Mycophenolate mofetil (CellCept) proved non-inferior to cyclophosphamide (Cytoxan) for treating ANCA-associated vasculitis in a regimen that permitted use of glucocorticoids, but not in o
There are many indications for plasma exchange in nephrology: some common ones include antibody-mediated rejection of a kidney transplant, Goodpastures, ANCA-associated vasculitis, and TTP-all of which are commonly associated with loss of renal fellows sleep-that is, you may…. ...
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Anti-Neutrophil antibody [NIMP-R14] (ab2557) has been cited in 70 publications. References for Human, Mouse in IF, IHC, IHC-Fr, IHC-P
·          As in ANCA vasculitis, avacopan demonstrated statistically significant improvement in renal function as measured by eGFR compared to placebo over 26 weeks of blinded treatment·          The change from...
TY - JOUR. T1 - ANCA-associated vasculitis after scrub typhus. AU - Kang, Yoon. AU - Jang, Hui Won. AU - Han, Sang Hoon. AU - Jeong, Su Jin. AU - Ku, Nam Su. AU - Baek, Ji Hyeon. AU - Kim, Chang Oh. AU - Choi, Jun Yong. AU - Song, Young Goo. AU - Lee, Sarah. AU - Park, Yong Beom. AU - Lee, Soo Kon. AU - Kim, Seung Min. AU - Kim, June Myung. PY - 2011/2. Y1 - 2011/2. N2 - Anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis is a primary systemic vasculitis that affects the small vessels, and ANCA is involved as the common pathogenesis. Environmental factors such as infectious agents have been considered to play a role in triggering the autoimmunity. We report here on a case of ANCA-associated vasculitis that developed after scrub typhus. A 64-year-old male was admitted because of fever, chills, pain, weakness and hypoesthesia of his calves. He was diagnosed as having scrub typhus based on the findings of an eschar and the positive serum anti-orientia antibody. The fever continued ...
TY - JOUR. T1 - Low serum complement 3 level is associated with severe ANCA-associated vasculitis at diagnosis. AU - Choi, Hyeok. AU - Kim, Youhyun. AU - Jung, Seung Min. AU - Song, Jason Jungsik. AU - Park, Yong Beom. AU - Lee, Sang Won. PY - 2019/2/15. Y1 - 2019/2/15. N2 - Objectives: We investigated whether low serum C3 level can cross-sectionally estimate severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in immunosuppressive drug-naïve patients at diagnosis. Methods: We retrospectively reviewed the medical records of 139 patients with AAV, who were first classified as AAV at Severance Hospital. We obtained clinical and laboratory data including serum complement 3 (C3) level and calculated Birmingham vasculitis activity score (BVAS) at diagnosis. We stratified AAV patients into three groups according to the tertile of BVAS and defined the lower limit of the highest tertile as the cutoff for severe AAV (BVAS at diagnosis ≥ 16) at diagnosis. Low serum C3 level was ...
Purchase ANCA-Associated Vasculitis, An Issue of Rheumatic Disease Clinics, Volume 36-3 - 1st Edition. Print Book. ISBN 9781437724936
Extracellular adenosine, generated via the concerted action of CD39 and CD73, contributes to T-cell differentiation and function. Adenosine concentrations are furthermore influenced by adenosine deaminase binding protein CD26. Because aberrant T-cell phenotypes had been reported in anti-neutrophil cytoplasmic auto-antibody (ANCA)-associated vasculitis (AAV) patients, an impaired expression of these molecules on T-cells of AAV patients was hypothesized in the present study. While in AAV patients (n = 29) CD26 was increased on CD4+ lymphocytes, CD39 and CD73 were generally reduced on patients T-cells. In CD4+ cells significant differences in CD73 expression were confined to memory CD45RA- cells, while in CD4- lymphocytes differences were significant in both naïve CD45RA+ and memory CD45RA- cells. The percentage of CD4-CD73+ cells correlated with micro-RNA (miR)−31 expression, a putative regulator of factor inhibiting hypoxia-inducible factor 1 alpha (FIH-1), inversely with serum C-reactive protein
Anti-neutrophil cytoplasmic antibody (ANCA) - associated vasculitis (AAV) is a life-threatening autoimmune disease characterized by an antibody-mediated glomerulonephritis and necrotizing vasculitis. Apart from antibodies, T cells are also involved in disease pathogenesis. This review stresses the hallmarks of T cell-mediated pathology in AAV and highlights the characteristics of lesional and circulating T cells in the immune response in AAV. Circulating effector T-cell populations are expanded and are in a persistent state of activation. Circulating regulatory T-cell subsets are less well characterized but seem to be impaired in function. Lesional effector T cells are present in granulomas, vasculitic lesions, and nephritis. Lesional T cells usually show pro-inflammatory properties and promote granuloma formation. Apart from T cells, dendritic cells are abundantly present at the sites of inflammation and locally orchestrate the immune response. Targeting the above-mentioned T cell-mediated disease
Avacopan is noninferior, but not superior, to prednisone with respect to remission at week 26 for patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis.
TY - JOUR. T1 - Interventions for renal vasculitis in adults. AU - Walters, Giles D.. AU - Willis, Narelle S.. AU - Cooper, Tess E.. AU - Craig, Jonathan C.. PY - 2020/1/13. Y1 - 2020/1/13. N2 - BACKGROUND: Renal vasculitis presents as rapidly progressive glomerulonephritis and comprises of a group of conditions characterised by acute kidney injury (AKI), haematuria and proteinuria. Treatment of these conditions involve the use of steroid and non-steroid agents in combination with plasma exchange. Although immunosuppression overall has been very successful in treatment of these conditions, many questions remain unanswered in terms of dose and duration of therapy, the use of plasma exchange and the role of new therapies. This 2019 publication is an update of a review first published in 2008 and updated in 2015. OBJECTIVES: To evaluate the benefits and harms of any intervention used for the treatment of renal vasculitis in adults. SEARCH METHODS: We searched the Cochrane Kidney and Transplant ...
Human Heat Shock Protein 60 (hHSP60) has been implicated in autoimmunity through molecular mimicry, based on the high degree of homology with HSP65 of micro-organisms leading to autoimmune recognition of the human protein. Additionally, sequence homology between hHSP60 and myeloperoxidase (MPO) has been described. MPO is a major autoantigen in vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA). We hypothesized that infections may trigger the ANCA response against MPO through hHSP60. In 86 consecutive patients with ANCA-associated vasculitis (AAV), anti-hHSP60 and anti-mycobacterial HSP65 were measured by ELISA. Patients were compared with 69 healthy controls (HC). Continuous data between groups were compared using Wilcoxon signed rank test and Kruskal-Wallis test with Dunns post-test when appropriate. Correlations between data were derived using Spearman correlation. Odds ratios and 95% confidence intervals were obtained using Fishers exact test. At diagnosis, median anti-mHSP65
Randomized, controlled, national, multicenter, prospective study to compare systematic rituximab infusions (conventional therapy) to rituximab infusion based on rate of ANCA and CD19 lymphocytes in patients with systemic ANCA-associated vasculitis, in remission (achieved with an induction treatment combining corticosteroids and an immunosuppressant after the first flare of the disease (new diagnosis) or after a relapse. Patients will be stratified by first flare (66% of the patients) or relapse (33% of the patients). Patients complying with the inclusion criteria may be included when they are in remission from their vasculitis. Patients will be included at the time of remission and then randomized. They will receive maintenance treatment by 1)2 rituximab infusions mg at D1, D15 then every 6 months until month 18 (i.e. a total of 5 infusions), at the dose of 500 mg. 2) 1 rituximab infusion at the dose of 500 mg at D0 then ANCA status and CD19+ lymphocyte count will be monitored every 3 months, ...
An elderly woman w/ history of IDA, aortic aneurysm + AI, and monoclonal gammopathy presents with SOB, found to have rapidly progressive anemia, AKI, and R,L pulmonary infiltrate concerning for a pulmonary-renal syndrome. She was ultimately diagnosed with ANCA vasculitis with +MPO, possibly related to hydralazine use and with some SLE overlap. Despite steroids and cytoxan, her renal failure progressed quickly requiring dialysis, and she ultimately transitioned to comfort care several months after the initial diagnosis ...
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Results We included 857 patients (77% women), age 18-85 years, disease duration 0-58 years. Persons with SLE were younger 47(34-57) than persons with SSc 60(52-69) p,0.001 and AAV 62(49-69) p,0.001, and they had longer disease duration 10 (2-20) than both the SSc 2 (0-8) p,0.001 and the AAV 3 (0-8) p,0.001 groups.. SLE patients reported a higher anxiety level and more impact of fatigue on all analysed subscales compared to the AAV patients, and all but impact on daily activities compared to SSc. SSc and AAV had a similar pattern on all analysed components (Table 1).. Among persons with disease duration less than a year, SLE still scored highest on all components of fatigue, but interestingly AAV patients reported similar figures to SLE regarding the severity and distress of fatigue (Table 2). ...
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Investigators have made a major advance in treating people with a severe form of vasculitis, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, a rare but devastating disease of blood...
Reactivity: Cow (Bovine), Dog (Canine) Host: Rabbit Clone: Polyclonal 1 image 1 PubMed reference | Order NCF4 antibody (ABIN2782428).
Stegeman, CA (Nov 2005). "Predictive value of antineutrophil cytoplasmic antibodies in small-vessel vasculitis: is the glass ... Anti-neutrophil cytoplasmic antibodies (ANCAs) are a group of autoantibodies, mainly of the IgG type, against antigens in the ... images of pANCA and cANCA fluorescence images of ANCA Anti-Neutrophil+Cytoplasmic+Antibody at the US National Library of ... Jennette, JC; Xiao, H; Falk, RJ (May 2006). "Pathogenesis of vascular inflammation by anti-neutrophil cytoplasmic antibodies". ...
Determination of anti-neutrophil cytoplasmic antibodies (ANCAs) can aid in the diagnosis, but positivity is not conclusive and ... Seo P, Stone JH (July 2004). "The antineutrophil cytoplasmic antibody-associated vasculitides". The American Journal of ... It is now widely presumed that the anti-neutrophil cytoplasmic antibodies (ANCAs) are responsible for the inflammation in GPA. ... Lally, L; Spiera, R (2015). "Current landscape of antineutrophil cytoplasmic antibody-associated vasculitis: classification, ...
Bosch X, Guilabert A, Espinosa G, Mirapeix E (2007). "Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a ... drug in relation to symptoms Palpable purpura Maculopapular rash Skin biopsy showing neutrophils around vessel IgA vasculitis ( ... A systematic review of antineutrophil cytoplasmic antibody-positive vasculitis identified best treatments depending on whether ... but not most other vasculitides. Antineutrophil cytoplasmic antibody may suggest granulomatosis with polyangiitis, microscopic ...
Several of these vasculitides are associated with antineutrophil cytoplasmic antibodies. These are: Granulomatosis with ... which is vascular damage caused by nuclear debris from infiltrating neutrophils. It typically presents as palpable purpura. ... Antineutrophil cytoplasmic antibody-associated vasculitides: is it time to split up the group? Ann Rheum Dis A Brooke W Eastham ... Other possible findings are elevated antineutrophil cytoplasmic antibody (ANCA) levels and hematuria. Other organ functional ...
... and antineutrophil cytoplasmic antibodies (ANCA) against neutrophil granulocytes. Two pathological subsets of EGPA are ... "Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review". JAMA. 298 (6): 655-69. doi: ... In addition to mepolizumab, a number of emerging targeted biotherapies-including the anti-IgE monoclonal antibody omalizumab, ... differentiated by the presence of antineutrophil cytoplasmic antibodies (ANCA), autoantibodies that mistakenly target and ...
Antibodies are also known as anti-neutrophil cytoplasmic antibodies (ANCAs), though ANCAs have also been detected in staining ... "Emerging concepts in the pathogenesis of antineutrophil cytoplasmic antibody-associated vasculitis". Current Opinion in ... Antibodies against MPO have been implicated in various types of vasculitis, most prominently three clinically and ... Neutrophil myeloperoxidase has a heme pigment, which causes its green color in secretions rich in neutrophils, such as mucus ...
... in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Treated Concomitantly With Rituximab or ... is a medication used to treat anti-neutrophil cytoplasmic autoantibody-associated vasculitis. The most common side effects ... in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis". ACR Open Rheumatol. 2 (11): 662-671. doi:10.1002/ ... In the United States, avacopan is indicated as an adjunctive treatment of adults with severe active anti-neutrophil cytoplasmic ...
It can also be classed as an autoimmune form of vasculitis. It is not associated with anti-neutrophil cytoplasmic antibodies, ... Kawasaki disease is also considered to be a primary childhood vasculitis, a disorder associated with vasculitis that mainly ... "necrotizing vasculitis - definition of necrotizing vasculitis". Free Online Medical Dictionary, Thesaurus and Encyclopedia. ... Under this classification scheme for systemic vasculitis, Kawasaki disease is considered to be a necrotizing vasculitis (also ...
... and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides such as granulomatosis with polyangiitis (GPA), ... Some examples of systemic vasculitic disease are: IgA vasculitis, Hypocomplementemic urticarial vasculitis, polyarteritis ... Some patients with systemic vasculitis will have their multi-organ disease spread to the peripheral nervous system; this is ... "Vasculitis and peripheral neuropathy:". Current Opinion in Rheumatology. 31 (1): 40-45. doi:10.1097/BOR.0000000000000559. ISSN ...
... anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, Graves' disease, myasthenia gravis, Addison's disease. The ... Treatment of PTPN22-deficient mice with an anti-GITR-L blocking antibody suppresses the expansion of Treg cells. PTPN22 ... Despite of those effects of the PTPN22 deficiency on a T cell compartment and an antibody production, PTPN22-deficient mice do ... PTPN22-deficient mice have increased levels of antibodies. However, there is no increase in levels of autoantibodies. ...
... , or MPO-ANCA, or perinuclear anti-neutrophil cytoplasmic antibodies, are antibodies that stain the material around the ... These include lactoferrin, elastase, and cathepsin G. When the condition is a vasculitis, the target is usually MPO. However, ... They are a special class of anti-neutrophil cytoplasmic antibodies. This pattern occurs because the vast majority of the ... Focal necrotizing and crescentic glomerulonephritis Rheumatoid arthritis C-ANCA Anti-neutrophil cytoplasmic antibody (ANCA) ...
... and pericarditis Intestinal necrosis and perforation PAN has no association with anti-neutrophil cytoplasmic antibodies, but ... Sarah Ringold; Carol A Wallace (May 1, 2010). "Evolution of paediatric-specific vasculitis classification criteria". Annals of ... Perinuclear pattern of antineutrophil cytoplasmic antibodies (p-ANCA) - not associated with "classic" polyarteritis nodosa, but ... Polyarteritis nodosa (PAN) is a systemic necrotizing inflammation of blood vessels (vasculitis) affecting medium-sized muscular ...
October 2002). "Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis ... Then, the large number of antibodies make it more likely that they will bind a neutrophil. Once bound, the neutrophil ... An important diagnostic test is the presence of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) with myeloperoxidase ... Most recently, two different groups of investigators have demonstrated that anti-MPO antibodies alone can cause necrotizing and ...
It is also the target of anti-neutrophil cytoplasmic antibodies (ANCAs) of the c-ANCA (cytoplasmic subtype) class, a type of ... Hilhorst M, van Paassen P, Tervaert JW (October 2015). "Proteinase 3-ANCA Vasculitis versus Myeloperoxidase-ANCA Vasculitis". ... Ohlsson K, Linder C, Rosengren M (July 1990). "Monoclonal antibodies specific for neutrophil proteinase 4. Production and use ... PRTN3 is a serine protease enzyme expressed mainly in neutrophil granulocytes. Its exact role in the function of the neutrophil ...
It is thought that antineutrophil cytoplasmic antibodies (ANCA) interact with antigens in the cytoplasm of neutrophils to cause ... Jennette, J. Charles; Nachman, Patrick H. (2017-10-06). "ANCA Glomerulonephritis and Vasculitis". Clinical Journal of the ... and type III and idiopathic RPGN are frequently associated with anti-neutrophil cytoplasmic antibodies (ANCA)-positive serum. ... The presence of anti-glomerular basement membrane (GBM) antibodies suggests type I RPGN; antinuclear antibodies (ANA) may ...
Zhang J, Zhang HY, Chen SZ, Huang JY (2016). "Anti-neutrophil cytoplasmic antibodies in cholesterol embolism: A case report and ... eosinophilic vasculitis, and eosinophilic ulcer of the oral mucosa. Other examples of organ-restricted hepereosinophilia ... Affected tissues exhibit acute inflammation involving eosinophils, neutrophils, monocytes, lymphocytes, and plasma cells. The ... nonsteroidal anti-inflammatory drugs (NSAIDs), some antipsychotics such as risperidone, and certain antibiotics. Phenibut, an ...
"Anti-Neutrophil Cytoplasmic Autoantibodies with Specificity for Myeloperoxidase in Patients with Systemic Vasculitis and ... In contrast, normal-density neutrophils were activated by MPO-ANCA and monoclonal anti-PR3 antibody. Normal-density neutrophil ... "Anti-Neutrophil Cytoplasmic Autoantibodies with Specificity for Myeloperoxidase in Patients with Systemic Vasculitis and ... "Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro". Proceedings ...
... the maker of Tavneos-a drug treatment for rare diseases called anti-neutrophil cytoplasmic autoantibody-associated vasculitis- ... In June 2015, Amgen presented Phase II clinical trial data for their anti-CGRP antibody AMG 334 for migraine, approved for sale ... would license its Phase II candidate drug AMG 714 to developer Celimmune who plan to develop the anti-IL-15 monoclonal antibody ... "Amgen bets up to $1.7B on Xencor's antibody technology". FierceBiotech. 16 September 2015. "Amgen, Xencor to Partner on Cancer ...
... of people Anti-double stranded DNA - 30% of people Atypical perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) Type 2 ... vasculitis, and autoimmune thyroiditis. The prevailing theory for the development of autoimmune hepatitis is thought to be the ... Anti-smooth muscle antibody (ASMA) - 65% of people Anti-actin antibodies Anti-mitochondrial antibodies - rare except for ... A number of specific antibodies found in the blood (antinuclear antibody (ANA), anti-smooth muscle antibody (SMA), anti-liver ...
The blot was reacted with anti-β-actin antibody (for other details of the blot see the reference ) The use of actin as an ... However the use of antibodies directed against different actin isoforms allows identifying not only the cytoplasmic beta in the ... Parks QM, Young RL, Poch KR, Malcolm KC, Vasil ML, Nick JA (Apr 2009). "Neutrophil enhancement of Pseudomonas aeruginosa ... vasculitis, and other conditions. Further, due to actin's close association with the apparatus of muscular contraction its ...
Nonlinear pharmacokinetics of rituximab in anti-neutrophil cytoplasmic antibody associated vasculitis. Denis Mulleman 1, 2 ... Nonlinear pharmacokinetics of rituximab in anti-neutrophil cytoplasmic antibody associated vasculitis.. EULAR 2019, Jun 2019, ...
It helps diagnose autoimmune vasculitis and inflammatory bowel disease. Learn more. ... This test looks for antineutrophil cytoplasmic antibodies (ANCA) in your blood. ... Other names: ANCA antibodies, cANCA, pANCA, cytoplasmic neutrophil antibodies, serum, anticytoplasmic autoantibodies ... What is an antineutrophil cytoplasmic antibodies (ANCA) test?. This test looks for antineutrophil cytoplasmic antibodies (ANCA ...
Access 704 top medical experts on Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis across 46 countries and 32 U.S. ... Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Group of systemic vasculitis with a strong association with anca. ... 704 top medical experts on Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis across 46 countries and 32 U.S. states, ... Find Expert Doctors on Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis. Based on Scientific Publications, NIH Grants ...
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Medicine & Life Sciences 100% * Antineutrophil Cytoplasmic ... keywords = "Antineutrophil cytoplasmic antibody-associated vasculitis, Biomarkers, Eosinophilic granulomatosis with ... promising biomarkers of disease activity and organ involvement in antineutrophil cytoplasmic antibody-associated vasculitis. ... promising biomarkers of disease activity and organ involvement in antineutrophil cytoplasmic antibody-associated vasculitis, ...
Markers for work disability in anti-neutrophil cytoplasmic antibody-associated vasculitis Basu N., McClean A., Harper L., Amft ...
High proteinase 3-anti-neutrophil cytoplasmic antibody (ANCA) level measured by the capture enzyme-linked immunosorbent assay ... Outcome of renal replacement therapy in antineutrophil cytoplasmic antibody-associated systemic vasculitis. J Am Soc Nephrol ... Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis. Ann ... focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis 2007;66:605-17. ...
What is Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis?. Related Articles. Mind-Altering Substances ... NSAIDs (non-steroidal anti-inflammatory drugs), SAIDs (steroidal anti-inflammatory drugs), and other alternative injective ... How Anti Inflammatory Oils Work To Reduce Joint Pain And Inflammation Naturally ...
... of small-to-medium-sized blood vessels and the presence of autoantibodies against cytoplasmic proteases sited in neutrophils ... Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) are characterized by inflammation ... Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) are characterized by inflammation of small-to- ... Phagocytotic function of macrophages can be impaired by surface expression of cytoplasmic proteases on apoptotic neutrophils ...
Its hallmark features include necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized ... Novel therapies for anti-neutrophil cytoplasmic antibody-associated vasculitis. Drugs. 2008. 68:747-770. [QxMD MEDLINE Link]. ... Antineutrophil cytoplasmic antibody testing. * Cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) directed against PR3 is ... Rituximab as maintenance therapy for anti-neutrophil cytoplasmic antibody-associated vasculitis. Clin J Am Soc Nephrol. 2010 ...
Propylthiouracil-induced anti-neutrophil cytoplasmic antibodies positive vasculitis. Alves Marta , Neves Celestino , Magalhaes ... The role of anti-DNA antibodies in pathogenesis of Hashimoto s thyroiditis. Vagapova Gulnar , Sattarova Lilya ... The objective of this study was to estimate the seroprevalence rates of anti-thyroid antibodies in HTLV-I carriers and HTLV-I- ... Recently it has been found that the HT patients demonstrate increased levels of anti-DNA antibodies, which may be directed ...
Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR ... so is renal vasculitis; glomerulonephritis due to vasculitis is never found; antineutrophil cytoplasmic antibodies (ANCA) are ... antineutrophil cytoplasmic antibodies, BVAS = Birmingham vasculitis activity score, CI = confidence intervals, CS = ... Antineutrophil cytoplasmic antibodies (ANCA) had been sought in 72/115 patients. The technique used followed the ...
... vasculitis syndromes (eg, Wegener granulomatosis), and infectious processes. In DPGN, mo... ... Lazarus B, John GT, OCallaghan C, Ranganathan D. Recent advances in anti-neutrophil cytoplasmic antibody-associated vasculitis ... For the initial treatment of of pauci-immune glomerulonephritis with or without circulating antineutrophil cytoplasmic antibody ... Anti-GBM Antibody-Induced Diffuse Proliferative Glomerulonephritis/Crescentic Glomerulonephritis. KDIGO guidelines recommend ...
... in the sera of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients and found that anti-PTX3 aAbs ... Clinical course of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and systemic vasculitis. The ... Vasculitis and glomerulonephritis: a subgroup with an antineutrophil cytoplasmic antibody. Aust N Z J Med. 1984 Jun. 14(3):277- ... Antineutrophil cytoplasmic antibodies and associated diseases: a review of the clinical and laboratory features. Kidney Int. ...
... anti-neutrophil cytoplasmic antibody) associated systemic vasculitis. The diseases present with acute inflammation in the small ...
ANCA: Anti-neutrophil cytoplasmic antibodies. The abbreviation is pronounced just like the last name of the singer, Paul Anka. ... These antibodies are found in patients with some forms of vasculitis, particularly Granulomatosis with polyangiitis (GPA/ ... The YouTube VF Education Channel features presentations, interviews and webinars by vasculitis experts defining vasculitis and ... The Vasculitis Clinical Research Consortium (VCRC) investigators provided the answers to the questions below. If you have a ...
Anti-neutrophil cytoplasmic antibody positive vasculitis. PT. Population Age: Adolescents, Under 18, Adults, Elderly. Gender: ... controlled trial comparing rituximab with azathioprine as maintenance therapy in relapsing ANCA-associated vasculitis. ...
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis 100% * Vascular Stiffness 80% * Fibrinolysis 76% ... and impaired fibrinolysis are pathogenic mechanisms contributing to cardiovascular risk in ANCA-associated vasculitis. Farrah, ...
Avacopan is in Phase III development for the treatment of anti-neutrophil cytoplasmic auto-antibody-associated vasculitis (AAV ... In addition to testing the effect of avacopan on improving active vasculitis, the ADVOCATE trial will also test the effect of ... ChemoCentryxs Phase III ADVOCATE trial of avacopan for the treatment of ANCA-associated vasculitis has surpassed 30 percent of ... grant revenue from the FDA to support the clinical development of avacopan for the treatment of patients with ANCA vasculitis. ...
Our Vasculitis Program is the first comprehensive, multispecialty vasculitis treatment program in Southern California. ... Antineutrophil cytoplasmic autoantibody (ANCA) vasculitis: Specific antibodies called ANCAs cause inflammation by attaching to ... Leukocytoclastic vasculitis (LCV): This type occurs when immune cells called neutrophils break down and cause inflammation. It ... Central nervous system vasculitis (CNS vasculitis): This type of vasculitis affects blood vessels in the brain and spine. ...
Phenomenon Systemic Vasculitis Anti-Neutrophil Cytoplasmic Antibody-Associated Churg-Strauss Syndrome Microscopic Polyangiitis ... Va sculitis C14.907.940.897.249.249 Churg-Strauss Syndrome C14.907.940.897.249.500 Microscopic ... Hyperplasia with ... Associated Vasculitis C14.907.940.897.249.249...........................................Churg-Strauss Syndrome C14.907.940.897. ...
Predictors of renal outcomes in sclerotic class anti-neutrophil cytoplasmic antibody glomerulonephritis. Am J Nephrol 48: 465- ... Kidney biopsy chronicity grading in antineutrophil cytoplasmic antibody associated vasculitis. Nephrol Dial Transplant gfab250 ... Histopathologic and clinical predictors of kidney outcomes in ANCA-associated vasculitis. Am J Kidney Dis 63: 227-235, 2014. ...
Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type. Ann ... and response to treatment with rituximab or cyclophosphamide in Anti-Neutrophil Cytoplasmic Antibody-Associated vasculitis. ... Kimberly RP (2015). The Road Traveled: Genomics and Biomarkers in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. ... 2015). Peripheral CD5+ B-cells in antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol, 67 (2), 535- ...
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis 100% * Antineutrophil Cytoplasmic Antibodies 86% * Antibody ... ANCA-associated vasculitis. Kitching, A. R., Anders, H. J., Basu, N., Brouwer, E., Gordon, J., Jayne, D. R., Kullman, J., Lyons ... ANCA-associated vasculitis in scleroderma: A case series of fourteen patients. Liang, K. P. & Michet, C. J., Apr 1 2011, In: ... ANCA-associated vasculitis-clinical utility of using ANCA specificity to classify patients. Cornec, D., Gall, E. C. L., ...
Screening for rheumatoid factor, antinuclear antibody, anti-extractable nuclear antigens, anti-neutrophil cytoplasmic and ... Anti-neutrophil cytoplasmic antibody-associated paraneoplastic vasculitis. Postgrad Med J. 1994 May; 70(823): 373-375. PubMed. ... Rituximab, the chimeric anti-CD20 antibody, is widely used in the clinical treatment of patients with NHL. This drug sensitizes ... Jazirehi AR, Huerta-Yepez S, Cheng G, Bonavida B. Rituximab (chimeric anti-CD20 monoclonal antibody) inhibits the constitutive ...
Markers for work disability in anti-neutrophil cytoplasmic antibody-associated vasculitis. Basu, N., McClean, A., Harper, L., ... Explaining fatigue in ANCA-associated vasculitis. Basu, N., McClean, A., Harper, L., Amft, E. N., Dhaun, N., Luqmani, R. A., ... Current evidence of anti-tumor necrosis factor α treatment efficacy in childhood chronic uveitis: a systematic review and meta- ... Aspirin and non-steroidal anti-inflammatory drug use and the risk of upper aerodigestive tract cancer. MacFarlane, T., Lefevre ...
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) Aromatic L-amino acid decarboxylase (AADC) deficiency ...
What is the role of rheumatoid factor positivity in ANCA-associated vasculitis? A new study investigates. ... Rheumatoid Factor Positivity in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. A Distinct Clinical Entity or ... Neutrophil count (/mm3). 8798.3 (4195.6). 5325.0 (3850.0-8275.0). ,0.001. Platelet count (×1000/mm3). 356.5 (269.0-477.0). ... Birmingham vasculitis activity score; C: cytoplasmic; EGPA: eosinophilic granulomatosis with polyangiitis; FFS: five factor ...
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis [C14.907.940.897.249] Anti-Neutrophil Cytoplasmic Antibody- ... Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis [C20.111.193] Anti-Neutrophil Cytoplasmic Antibody-Associated ... ANCA-Associated Vasculitis Anti Neutrophil Cytoplasmic Antibody Associated Vasculitis Pauci Immune Vasculitis Pauci-Immune ... ANCA-Associated Vasculitis. Anti Neutrophil Cytoplasmic Antibody Associated Vasculitis. Pauci Immune Vasculitis. Pauci-Immune ...
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (14) * COVID-19 (12) * Genetic Predisposition to Disease (11) ... Sensitivity of SARS-CoV-2 B.1.1.7 to mRNA vaccine-elicited antibodies. Collier, Dami A; De Marco, Anna; Ferreira, Isabella A T ... Author Correction: Sensitivity of SARS-CoV-2 B.1.1.7 to mRNA vaccine-elicited antibodies. Collier, Dami A; De Marco, Anna; ...
  • ChemoCentryx recently received orphan designation in Switzerland from SwissMedic for avacopan for the treatment of two forms of ANCA-vasculitis: microscopic polyangiitis and granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis). (globenewswire.com)
  • TAVNEOS ® (avacopan) is indicated as an adjunctive treatment of adult patients with severe active anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (granulomatosis with polyangiitis [GPA] and microscopic polyangiitis [MPA]) in combination with standard therapy including glucocorticoids. (amgen.com)
  • ANCA-associated vasculitis is a chronic inflammatory disease of the blood vessel wall that can be subclassified into granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis, the investigators explain. (medwirenews.com)
  • [ 1 ] These anti-GBM antibodies produce a characteristic linear deposition along the GBM, one way in which Goodpasture syndrome is differentiated from Wegener granulomatosis. (medscape.com)
  • Because pulmonary renal syndrome is discussed extensively elsewhere (see Wegener Granulomatosis ), this article focuses on the specific form of this syndrome associated with anti-GBM antibodies. (medscape.com)
  • We undertook a systematic literature review as a background to the European League Against Rheumatism (EULAR) recommendations for conducting clinical trials in anti-neutrophil cytoplasm antibody associated vasculitis (AAV), and to assess the quality of evidence for outcome measures in AAV. (bmj.com)
  • Glucocorticoid treatment and damage in the anti-neutrophil cytoplasm antibody-associated vasculitides: long-term data from the European Vasculitis Study Group trials. (medscape.com)
  • Nomenclature of systemic vasculitides. (medscape.com)
  • Primary systemic vasculitides (PSV) refer to a group of chronic autoimmune disorders affecting various-sized vessels. (medscape.com)
  • [ 1 , 2 ] Among the primary systemic vasculitides, ANCA-associated vasculitis (AAV) is characterized by necrotizing inflammation of the small- and medium-sized vessels, which is typically associated with the production of ANCAs against MPO or proteinase 3 (PR3). (medscape.com)
  • Specific antibodies called ANCAs cause inflammation by attaching to immune cells called neutrophils. (uclahealth.org)
  • A meta-analysis has highlighted the cardiovascular (CV) risk faced by patients with vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCAs). (medwirenews.com)
  • Cyclophosphamide and glucocorticoids have reduced mortality in antineutrophil cytoplasm antibody (ANCA) associated vasculitides (AAV), although cure remains uncommon. (bmj.com)
  • Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) are characterized by inflammation of small-to-medium-sized blood vessels and the presence of autoantibodies against cytoplasmic proteases sited in neutrophils and monocytes. (nih.gov)
  • Nonlinear pharmacokinetics of rituximab in anti-neutrophil cytoplasmic antibody associated vasculitis. (univ-brest.fr)
  • In 2011, however, the US Food and Drug Administration (FDA) approved the use of rituximab (a monoclonal antibody that targets B cells), in combination with glucocorticoids, as an alternative to cyclophosphamide for induction of remission in AAV (GPA and microscopic polyangiitis). (medscape.com)
  • It is characterized by inflammation of the blood vessels (VASCULITIS) leading to damage in any number of organs. (ucdenver.edu)
  • Simon and colleagues investigated the presence of anti-pentraxin 3 (PTX3) autoantibodies (aAbs) in the sera of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients and found that anti-PTX3 aAbs were present in nearly 40% of patients studied, including in patients without detectable MPO and PR3 ANCA. (medscape.com)
  • The pathogenesis of anti-glomerular basement membrane (anti-GBM) disease (Goodpasture disease) is linked to the presence of autoantibodies that react with the alveolus in the lung and the basement membrane of the glomerulus in the kidney. (medscape.com)
  • Anti-GBM autoantibodies that are present in the circulation of patients with anti-GBM disease cross the fenestrated endothelium in the glomerulus and bind with the underlying GBM, inducing renal injury. (medscape.com)
  • The American College of Rheumatology 1990 criteria for the classification of vasculitis. (medscape.com)
  • Reporting in Rheumatology , Tiny Hoekstra (VU University Medical Centre, Amsterdam) and colleagues maintain: "There is a clear need for active cardiovascular risk management in patients with ANCA-associated vasculitis. (medwirenews.com)
  • UCLA Health specialists offer comprehensive care for vasculitis, a rare autoimmune condition that causes inflammation. (uclahealth.org)
  • Vasculitis is a rare group of conditions that causes inflammation throughout the body. (uclahealth.org)
  • Vasculitis is a rare group of conditions in which the immune system attacks the blood vessels, causing inflammation. (uclahealth.org)
  • Depending on which part of your body is affected by inflammation, vasculitis complications can be serious and potentially life-threatening. (uclahealth.org)
  • Leukocytoclastic vasculitis (LCV) is an immune-complex related vascular disorder that combines segmental inflammation with necrosis of the blood vessels. (cdlib.org)
  • Severe active ANCA-associated vasculitis therapy TAVNEOS ® becomes the newest medicine in Amgen's inflammation portfolio after close of ChemoCentryx deal. (amgen.com)
  • Last year's FDA approval of TAVNEOS, and now Amgen's involvement in progressing this first-in-class treatment as part of its inflammation portfolio, represent significant progress for the vasculitis community. (amgen.com)
  • TAVNEOS is an important evolution in the standard of care as the only FDA-approved targeted therapy for severe active ANCA-associated vasculitis," says Kave Niksefat, vice president and general manager, Inflammation, at Amgen. (amgen.com)
  • Chimeric murine/human monoclonal antibody directed against the CD20 antigen found on surface of B-lymphocytes. (medscape.com)
  • Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology? (medscape.com)
  • Anti-GBM disease is defined as the triad of glomerulonephritis (usually rapidly progressive or crescentic), pulmonary hemorrhage, and anti-GBM antibody formation. (medscape.com)
  • On the basis of this clinical report, Goodpasture's name is often linked to the pulmonary renal syndrome of alveolar hemorrhage and necrotizing and proliferative glomerulonephritis, although vasculitis and not anti-GBM disease is believed to be the cause of the pulmonary renal syndrome in Goodpasture's original patient. (medscape.com)
  • This autoimmune disease is characterized by necrotizing vasculitis that mainly affects small- to medium-sized blood vessels. (amgen.com)
  • We report 2 new cases of leishmaniasis involving patients with autoimmune rheumatic diseases who received anti-tumor necrosis factor (anti-TNF) agents. (cdc.gov)
  • We then searched Medline, EMBASE, and Current Contents databases for all reports on leishmaniasis in Europe and the Mediterranean area among patients with autoimmune rheumatic diseases, which are often treated with anti-TNF agents. (cdc.gov)
  • ChemoCentryx's Phase III ADVOCATE trial of avacopan for the treatment of ANCA-associated vasculitis has surpassed 30 percent of its target patient enrollment with more than 185 sites activated. (globenewswire.com)
  • In addition to testing the effect of avacopan on improving active vasculitis, the ADVOCATE trial will also test the effect of avacopan on preventing a recurrence of vasculitis. (globenewswire.com)
  • This designation is in addition to the previously received orphan designations from the U.S. Food and Drug Administration (FDA) and European Medicines Agency (EMA) for avacopan to treat ANCA-vasculitis. (globenewswire.com)
  • These increases were partially offset by lower grant revenue from the FDA to support the clinical development of avacopan for the treatment of patients with ANCA vasculitis. (globenewswire.com)
  • The increase in research and development expenses from 2016 to 2017 was primarily attributable to the initiation and patient enrollment of the avacopan Phase III ADVOCATE trial in patients with ANCA vasculitis and start-up expenses for the Phase II clinical trial of avacopan for the treatment of C3G. (globenewswire.com)
  • These increases were partially offset by lower costs associated with the completion of the avacopan CLEAR and CLASSIC Phase II clinical trials for the treatment of ANCA vasculitis and enrollment completion of the CCX872 Phase I trial in patients with advanced pancreatic cancer in 2016. (globenewswire.com)
  • TAVNEOS (avacopan) is a medicine approved by the FDA as an adjunctive treatment for adults with severe active antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, a rare disease. (amgen.com)
  • Antinuclear antibody (ANA) titer: The ANA titer result is not positive in patients with ANCA-associated disease. (medscape.com)
  • 2021). Evolution of epitope-specific IgE and IgG 4 antibodies in children enrolled in the LEAP trial . (immunetolerance.org)
  • Outcome measures in primary small vessel vasculitis help to describe the natural history of treated disease. (bmj.com)
  • Results of indirect immunoflorescent antibody (IFA) testing were positive for Leishmania parasites (titer 6,400). (cdc.gov)
  • The examination of Giemsa-stained smears from bone marrow aspirate demonstrated abundant Leishmania parasites, and IFA was marginally positive for Leishmania antibodies (titer 400). (cdc.gov)
  • One hundred fifteen patients were included in therapeutic trials organized by the French Vasculitis Study Group and/or referred to our department for HBV-PAN between 1972 and 2002. (lww.com)
  • The membership is especially valuable for vasculitis patients because their diseases are rare and present unique challenges due to disease damage and medication effects. (vasculitisfoundation.org)
  • We teach other specialists to recognize and evaluate patients with suspected vasculitis early so they can receive timely treatment. (uclahealth.org)
  • We know the challenges these specialists face and we will be able to help them treat severe active ANCA-associated vasculitis regardless of where their patients are in their journey with this debilitating disease. (amgen.com)
  • Amgen has also committed to assisting advocacy groups like the Vasculitis Foundation and others in the ANCA-associated vasculitis community by working with leading experts and creating resources to help to reach and educate both patients and the specialists who care for them. (amgen.com)
  • To investigate the significance of RF positivity in ANCA-associated vasculitis (AAV) patients. (medscape.com)
  • [ 9 ] Further, there are reports suggesting that RF is also frequently found in the peripheral circulation of patients with systemic vasculitis. (medscape.com)
  • [ 11 ] We have also demonstrated that RF positivity was associated with cutaneous manifestation in EGPA patients at the time of diagnosis, suggesting that this antibody could have clinical relevance in AAV and should be further investigated. (medscape.com)
  • Alternative steroid-sparing anti-inflammatory therapies, selected from among the options noted in the Medication section, should be considered in patients requiring long-term corticosteroid therapy. (medscape.com)
  • Characteristically, patients with severe systemic vasculitis have a poor response to the initial phases of treatment. (medscape.com)
  • The outlook for patients with severe systemic vasculitis is guarded because they have a considerable risk for dependent existence and progressive decline or premature death. (medscape.com)
  • The overall mortality rate may be as high as 25% within 5 years of diagnosis, half of these patients dying directly from vasculitis and half from secondary complications of vasculitis. (medscape.com)
  • Dutch researchers report that patients with ANCA-associated vasculitis had a 65% increased risk for CV events relative to the general population, similar to that observed in the setting of other chronic inflammatory diseases. (medwirenews.com)
  • To investigate further, the team conducted a meta-analysis of seven observational studies, including nearly 14,000 patients with ANCA-associated vasculitis who were compared with the general population in six studies and patients with chronic kidney disease in one study. (medwirenews.com)
  • They say their results underline recent guidelines from EULAR, which recommend active monitoring and treatment of traditional CV risk factors as part of the standard care for patients with ANCA-associated vasculitis. (medwirenews.com)
  • Reports increased during 2004-2008, especially for patients treated with anti-tumor necrosis factor agents. (cdc.gov)
  • Despite this triad of clinical findings, patients with anti-GBM disease may present with a spectrum of conditions ranging from pulmonary hemorrhage with minimal or no renal involvement to full-blown renal failure with limited or no pulmonary involvement. (medscape.com)
  • In this study, we used targeted serum proteomics to identify circulating biomarkers for prediction of disease activity and organ involvement in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). (elsevier.com)
  • The most important requirement in the diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated disease is a high index of suspicion. (medscape.com)
  • The Patient Information Book includes general information on vasculitis, the individual diseases, medications, tips for managing the disease and other resources. (vasculitisfoundation.org)
  • For people living with severe active ANCA-associated vasculitis, treatment is vital for achieving disease remission. (amgen.com)
  • People with severe active ANCA-associated vasculitis can be classified by their healthcare providers as in remission, and yet still be greatly impacted by this serious disease, with the potential to benefit from additional treatment," says Joyce Kullman, executive director, Vasculitis Foundation. (amgen.com)
  • The Vasculitis Foundation looks forward to partnering with Amgen on expanding awareness of this devastating rare disease," Kullman adds. (amgen.com)
  • ANCA-associated vasculitis carried a relative risk of 1.65 for all CV events, with a relative risk of 1.60 for ischemic heart disease. (medwirenews.com)
  • Goodpasture's name has been used in a more specific clinical condition known as Goodpasture disease, which is the pulmonary renal syndrome specifically associated with anti-glomerular basement membrane (anti-GBM) antibodies. (medscape.com)
  • To avoid confusion between Goodpasture syndrome and Goodpasture disease, the term anti-GBM disease is used. (medscape.com)
  • When it comes to rare and complex diseases like ANCA-associated vasculitis, it's not enough to simply have a medicine like TAVNEOS on the market. (amgen.com)
  • Although premature atherosclerosis and CV events are a well-established long-term complication of many inflammatory diseases, they note that the increased risk in ANCA-associated vasculitis is poorly quantified. (medwirenews.com)
  • The discovery of the role of anti-GBM antibodies by Lerner et al in 1967 helped provide both a better understanding of the pathogenesis for this specific form of pulmonary renal syndrome and a more rational approach to treatment. (medscape.com)
  • The Vasculitis Foundation is committed to providing the most current information on diagnosis, treatment and research efforts. (vasculitisfoundation.org)
  • Most of the time, doctors don't find a clear cause of vasculitis. (uclahealth.org)
  • A B-cell non-Hodgkin lymphoma was discovered in association with a severe leukocytoclastic vasculitis and disseminated intravascular coagulation. (cdlib.org)
  • TAVNEOS is an oral medicine that is thought to work by blocking certain receptors on destructive inflammatory cells - C5a - known to be a driver of severe active ANCA-associated vasculitis and its progression. (amgen.com)
  • Our goal is to keep improving the quality of care and access to treatment that people with severe active ANCA-associated vasculitis need and deserve. (amgen.com)
  • It's important to treat vasculitis early to lower your risk of these complications. (uclahealth.org)
  • Treatment-related complications include those associated with immunosuppression (eg, risk for infection) and other effects of anti-inflammatory medications such as Cushing ulcer with or without gastrointestinal perforation. (medscape.com)
  • This type of vasculitis affects blood vessels in the brain and spine. (uclahealth.org)
  • Its principal function is to inactivate neutrophil elastase, preventing tissue damage. (scielo.br)
  • The researchers suggest several pathophysiological mechanisms could account for the increased risk for CV events identified, such as active vasculitis, atherosclerosis, a hypercoagulable state, or hemorrhage. (medwirenews.com)
  • The YouTube VF Education Channel features presentations, interviews and webinars by vasculitis experts defining vasculitis and explaining diagnostic procedures, treatment options and the latest in research efforts. (vasculitisfoundation.org)
  • Call 310-582-6350 to learn more about vasculitis treatment at UCLA Health. (uclahealth.org)
  • The UCLA Health Vasculitis Program is the first comprehensive, multispecialty program dedicated to vasculitis treatment in Southern California. (uclahealth.org)
  • 2 years after the new anti-TNF treatment, he is well, with no signs or symptoms of leishmaniasis. (cdc.gov)
  • Building upon the collective strength of the vasculitis community, the Foundation supports, inspires and empowers individuals with vasculitis and their families through a wide range of education, research, clinical, and awareness initiatives. (vasculitisfoundation.org)
  • Our physicians raise awareness about vasculitis by educating trainees, physicians and allied health professionals. (uclahealth.org)
  • These are rare conditions, and most forms of vasculitis affect only several people out of a million. (uclahealth.org)