Vasculitis: Inflammation of any one of the blood vessels, including the ARTERIES; VEINS; and rest of the vasculature system in the body.Systemic Vasculitis: A heterogeneous group of diseases characterized by inflammation and necrosis of the blood vessel walls.Retinal Vasculitis: Inflammation of the retinal vasculature with various causes including infectious disease; LUPUS ERYTHEMATOSUS, SYSTEMIC; MULTIPLE SCLEROSIS; BEHCET SYNDROME; and CHORIORETINITIS.Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: 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.Vasculitis, Central Nervous System: 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)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.Churg-Strauss Syndrome: 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.Skin Diseases, Vascular: Skin diseases affecting or involving the cutaneous blood vessels and generally manifested as inflammation, swelling, erythema, or necrosis in the affected area.Rheumatoid Vasculitis: 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.Microscopic Polyangiitis: 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.Purpura, Schoenlein-Henoch: 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.Cryoglobulinemia: 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.Arteritis: INFLAMMATION of any ARTERIES.Mucocutaneous Lymph Node Syndrome: 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.Behcet Syndrome: 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.Glomerulonephritis: 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.Cryoglobulins: Abnormal immunoglobulins, especially IGG or IGM, that precipitate spontaneously when SERUM is cooled below 37 degrees Celsius. It is characteristic of CRYOGLOBULINEMIA.Giant Cell Arteritis: 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)Purpura: 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).Takayasu Arteritis: 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.Autoimmune Diseases: Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides.Immunosuppressive Agents: 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.Autoantibodies: Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.Mercuric Chloride: Mercury chloride (HgCl2). A highly toxic compound that volatizes slightly at ordinary temperature and appreciably at 100 degrees C. It is corrosive to mucous membranes and used as a topical antiseptic and disinfectant.Leg Dermatoses: A nonspecific term used to denote any cutaneous lesion or group of lesions, or eruptions of any type on the leg. (From Stedman, 25th ed)Plasmapheresis: 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.Urticaria: A vascular reaction of the skin characterized by erythema and wheal formation due to localized increase of vascular permeability. The causative mechanism may be allergy, infection, or stress.Prednisolone: 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.Biopsy: Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.Lupus Erythematosus, Systemic: 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.Antigen-Antibody Complex: 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.Temporal Arteries: Arteries arising from the external carotid or the maxillary artery and distributing to the temporal region.Antibodies, Monoclonal, Murine-Derived: Antibodies obtained from a single clone of cells grown in mice or rats.Cogan Syndrome: A condition consisting of inflammatory eye disease usually presenting as interstitial KERATITIS, vestibuloauditory dysfunction, and large- to medium-vessel vasculitis.Methylprednisolone: A PREDNISOLONE derivative with similar anti-inflammatory action.Plasma Exchange: 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.Arthus Reaction: 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.Cyclophosphamide: 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.Cecal Diseases: Pathological developments in the CECUM.Serine Endopeptidases: Any member of the group of ENDOPEPTIDASES containing at the active site a serine residue involved in catalysis.Skin DiseasesArthritis, Rheumatoid: A chronic systemic disease, primarily of the joints, marked by inflammatory changes in the synovial membranes and articular structures, widespread fibrinoid degeneration of the collagen fibers in mesenchymal tissues, and by atrophy and rarefaction of bony structures. Etiology is unknown, but autoimmune mechanisms have been implicated.Immune Complex Diseases: Group of diseases mediated by the deposition of large soluble complexes of antigen and antibody with resultant damage to tissue. Besides SERUM SICKNESS and the ARTHUS REACTION, evidence supports a pathogenic role for immune complexes in many other IMMUNE SYSTEM DISEASES including GLOMERULONEPHRITIS, systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC) and POLYARTERITIS NODOSA.Rheumatoid Factor: Antibodies found in adult RHEUMATOID ARTHRITIS patients that are directed against GAMMA-CHAIN IMMUNOGLOBULINS.Azathioprine: 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)Fever of Unknown Origin: Fever in which the etiology cannot be ascertained.Adrenal Cortex HormonesImmunoglobulins, Intravenous: 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.Neutrophils: 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.Peripheral Nervous System Diseases: Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves.Immunoglobulin G: The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.Pulse Therapy, Drug: Administration of high doses of pharmaceuticals over short periods of time.Immunologic Factors: Biologically active substances whose activities affect or play a role in the functioning of the immune system.Scleritis: 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.Necrosis: 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.Fatal Outcome: Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept.Glucocorticoids: 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.

*  Overview of the management of vasculitis in adults

The vasculitides are defined by the presence of leukocytes in the vessel wall with reactive damage to mural structures. In ... Vasculitis treatment: is it time to change the standard of care for ANCA-associated vasculitis? Presse Med 2013; 42:643. ... Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM 1994; 87:671. ... but significant overlap is observed among the vasculitides. The wide clinical spectrum of disease in the vasculitides presents ...

*  What's Up Doc? Early treatment affects vasculitis - News - The Herald News, Fall River, MA - Fall River, MA

Vasculitis is inflammation of a blood vessel(s). The symptoms and complications that occur from vasculitis depend on which ... Vasculitis is inflammation of a blood vessel(s). The symptoms and complications that occur from vasculitis depend on which ... Vasculitis is inflammation of a blood vessel(s). The symptoms and complications that occur from vasculitis depend on which ... What's Up Doc? Early treatment affects vasculitis. Tuesday. Sep 25, 2007 at 12:01 AM Sep 25, 2007 at 3:50 PM ...

*  Vasculitis UK Christmas cards - Vasculitis UK | HealthUnlocked

Here at vasculitis UK we are selling our Christmas cards, which have been hand drawn by our very own shop... ... Christmas Greetings from the Trustees of Vasculitis UK. *. Christmas Craft & Food Fair for Vasculitis UK - Sunday 15th November ... for more info, or email for orders. ... Here at vasculitis UK we are selling our Christmas cards, which have been hand drawn by our very own shop assistant, with each ...

*  British Library EThOS: The development of a humanised mouse model of ANCA associated vasculitis

Thus, this work has identified GCSF and phosphoinositol-3-kinase p/8 as molecules of potential importance in ANCA vasculitis. ... to establish a humanised mouse model of ANCA vasculitis. ANCA were purified from patient plasma, and two human neutrophil ... and are associated with a systemic autoimmune vasculitis, in which a pauci-immune crescentic glomerulonephritis is common. The ...

*  BioPlex 2200 Vasculitis | Clinical Diagnostics | Bio-Rad

... associated with systemic small vessel vasculitis on the BioPlex 2200 system ... BioPlex® 2200 Vasculitis Control CD 663-1840 CD with software to check performance of controls in vasculitis testing on BioPlex ... BioPlex® 2200 Vasculitis Calibrator CD 663-1820 CD with software to verify calibration of vasculitis screening on BioPlex® 2200 ... BioPlex® 2200 Vasculitis Calibrator Set 663-1800 Multiplexed standard analytes for calibration of results of vasculitis testing ...

*  Vasculitis Affecting Large Arteries

The hallmark of pathology associated with large vessel vasculitides is that chronic inflammatory lesions are primarily found ... The two main large artery vasculitides that will be discussed below are giant cell arteritis (GCA) and Takayasu arteritis (TA). ... The hallmark of pathology associated with large vessel vasculitides is that chronic inflammatory lesions are primarily found ...

*  One-Time DNA Study for Vasculitis - Full Text View -

Vasculitis. Systemic Vasculitis. Vasculitis, Central Nervous System. Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ... Each type of vasculitis is a rare ('orphan') disease. However, taken together, vasculitis affects tens of thousands of ... The Vasculitis Clinical Research Consortium (VCRC) currently focuses on 6 specific types of vasculitis that were selected to ... One-Time DNA Study for Vasculitis. This study is currently recruiting participants. See Contacts and Locations ...

*  Search of: Vasculitis Syndromes of the Central and Peripheral Nervous Systems - Results by Topic -

IMPORTANT: Listing of a study on this site does not reflect endorsement by the National Institutes of Health. Talk with a trusted healthcare professional before volunteering for a study. Read more... ...

*  Rhinologic and sinonasal changes in PR3 ANCA pulmonary vasculitis.

... patients with PR3 ANCA pulmonary vasculitis present with upper respiratory tract symptoms or sings. Nasal cavity usually ... BACKGROUND: Over 70-95% patients with PR3 ANCA pulmonary vasculitis present with upper respiratory tract symptoms or sings. ... Maxillary sinuses are regions which are most frequently affected during the course of PR3 ANCA pulmonary vasculitis. CT ... aim of the study was to characterize pathologies of nasal and sinonasal CT scans in patients with PR3 pulmonary ANCA vasculitis ...

*  Vasculitis | Arthritis Research UK

Vasculitis means inflammation of the blood vessels. There are many different types of the condition. Symptoms vary, but it can ... Who gets vasculitis?. Vasculitis is quite rare. Overall, in every 100,000 people in the UK, only 10-15 will develop vasculitis ... What causes vasculitis?. There's no single cause of vasculitis, and in most cases the exact cause is unknown. It's thought that ... What is vasculitis?. Vasculitis means inflammation of any of your blood vessels. It can cause a variety of different symptoms ...

*  Peroxiredoxin 2 is a novel autoantigen for anti-endothelial cell antibodies in systemic vasculitis.

... have been frequently detected in systemic vasculitis, which affects blood vessels of various sizes. To understand the ... was significantly higher in systemic vasculitis (60\%) compared to those in collagen diseases without clinical vasculitis (7 ... To understand the pathogenic roles of AECA in systemic vasculitis, we attempted to identify target antigens for AECA ... Abstract Anti-endothelial cell antibodies (AECA) have been frequently detected in systemic vasculitis, which affects blood ...

*  What Causes Vasculitis? - NHLBI, NIH

What Causes Vasculitis?. Vasculitis occurs if your immune system attacks your blood vessels by mistake. What causes this to ... You can have these disorders for years before developing vasculitis.. Vasculitis also may be linked to certain blood cancers, ... Sometimes an autoimmune disorder triggers vasculitis. Autoimmune disorders occur if the immune system makes antibodies ( ...

*  UNC Scientists Author Editorial On International Trials For Vasculitis - Redorbit

A different B-cell protein is the target of vasculitis.. Ronald J. Falk, MD, professor of medicine and director of the UNC ... All of more than 15 types of vasculitis are classified as rare diseases. In the U.S., a rare disease -- also called an orphan ... Vasculitis develops when blood vessels become inflamed, causing damage to tissue and organs. At present, the causes of ... In 1988, Falk and Jennette characterized one of the markers for vasculitis found in blood serum, and Falk leads the UNC ...

*  Other forms of vasculitis and pseudovasculitis.

Previous Document: Vasculitis associated with connective tissue disease.. Next Document: Vasculitis by organ systems.. ... Vasculitis / classification*, diagnosis, drug therapy, surgery. From MEDLINE®/PubMed®, a database of the U.S. National Library ... These conditions should always be kept in mind because their pathophysiology and treatment are different from true vasculitides ... In some patients the diagnosis of vasculitis precedes that of familial Mediterranean fever. Kawasaki disease, although rare, ...

*  Vasculitis 2013

... 模組 Vasculitis 魏正宗 中山醫學大學附設醫院過敏免疫風濕科主任 Tel: 04 ... Vasculitis 2013 * 1. 醫學系「免疫與感染學 V 」模組 Vasculitis 魏正宗 中山醫學大學附設醫院過敏免疫風濕科主任 Tel: 04 24729595 ext 34314; Email: ... Microscopic polyangiitis (Leukocytoclastic vasculitis) * 31. IgA Vasculitis (Henoch-Schönlein

*  Vino for Vasculitis! | Jessica Foster's Fundraiser

... help us commemorate her memory and touch so many others through raising awareness and money for the Vasculitis Foundation. You ... can read about her journey with vasculitis from her caring bridge: And check ... help us commemorate her memory and touch so many others through raising awareness and money for the Vasculitis Foundation. ...

*  Identification of an autoantibody to vascular endothelial cell-specific antigens in patients with systemic vasculitis.

Although immunologic mechanisms have been postulated in the pathogenesis of vasculitis, an autoimmune process directed against ... PATIENTS AND METHODS: The study patients included 21 with systemic vasculitis and four with hypersensitivity vasculitis. A ... RESULTS: An autoantibody to VEC antigens was detected in 18 of the 21 patients (86\%) with confirmed systemic vasculitis, not ... We examined the role of an immunologic response to vascular endothelial cell (VEC) antigens in patients with vasculitis, ...

*  In vivoapproaches to investigate ANCA-associated vasculitis: lessons and limitations | Arthritis Research & Therapy | Full Text

This raises the fundamental question of whether the pathogenesis of MPO-ANCA vasculitis and that of Pr3-ANCA vasculitis are the ... Strategies to develop Pr3-ANCA vasculitis models, in contrast to MPO-ANCA vasculitis models, have been unsuccessful so far. ... Overview of the experimental autoimmune vasculitis rat model of anti-myeloperoxidase (anti-MPO)-associated systemic vasculitis ... Experimental autoimmune vasculitis: an animal model of anti-neutrophil cytoplasmic autoantibody-associated systemic vasculitis ...

*  Plasma Exchange and Glucocorticoids for Treatment of Anti-Neutrophil Cytoplasm Antibody (ANCA) - Associated Vasculitis - Full...

Vasculitis. Granulomatosis with Polyangiitis. Microscopic Polyangiitis. Wegener's. ANCA-Associated Vasculitis. GPA. MPA. ... Vasculitis. Systemic Vasculitis. Granulomatosis with Polyangiitis. Microscopic Polyangiitis. Vascular Diseases. Cardiovascular ... Pulmonary hemorrhage due to active vasculitis defined by:. *A compatible chest x-ray or CT scan (diffuse pulmonary infiltrates) ... MedlinePlus related topics: Steroids Vasculitis Genetic and Rare Diseases Information Center resources: Granulomatosis With ...

*  Systemic Vasculitis During the Course of Systemic Sclerosis: Report of 12 Cases and Review of the Literature.

... the association of SSc and systemic vasculitis has rarely been described. We obtained information on cases of ... We obtained information on cases of systemic vasculitis associated with SSc in France from the French Vasculitis Study Group ... 9 with ANCA-associated systemic vasculitis (AASV) and 3 with mixed cryoglobulinemia vasculitis (MCV). In all AASV patients, SSc ... The characteristics of AASV were microscopic polyangiitis (n = 7) and renal limited vasculitis (n = 2). Anti-myeloperoxidase ...

*  IMPROVE: Mycophenolate Mofetil Versus Azathioprine for Maintenance Therapy in ANCA Associated Systemic Vasculitis

... The aim of IMPROVE is to define the optimal maintenance therapy for ANCA-associated vasculitides (AASV) by ... renal limited vasculitis (RLV), are progressive, multisystem, autoimmune diseases which require the prescription of ... The aim of IMPROVE is to define the optimal maintenance therapy for ANCA-associated vasculitides (AASV) by comparing the AZA ( ... Anti-Cytokine Therapy for Vasculitis. The purpose of this study is to determine whether Infliximab (monoclonal anti-tumour ...

*  Vasculitis

V 6 Small Vessel Vasculitis (Drug-induced). This low power photomicrograph of a deep skin biopsy shows how this vasculitis ... Vasculitis. V 1 Giant Cell (Temporal) Arteritis. This medium power photomicrograph shows a florid example of giant cell ... Often, patients with vasculitis may hemorrhage from the involved vessels, leading to clinically evident purpura.. V 7 Small ... but the histopathologic findings are identical to small vessel vasculitis due to other causes.. ...

*  Systemic Vasculitis

Web-based systemic vasculitis learning tool for residents during rheumatology rotation and self-study for rheumatology health ... Large-vessel vasculitis. Arthritis Rheum 2004;51:128-39.. * Seo P, Stone JH. Small-vessel and medium-vessel vasculitis. ... vasculitis secondary to connective tissue disorders. *vasculitis secondary to viral infection, including hepatitis B and ... Vasculitis mimics. Curr Opin Rheumatol 2008;20:29-34.. * Langford CA. Chronic immunosuppressive therapy for systemic vasculitis ...

Bullous small vessel vasculitis: Bullous small vessel vasculitis (also known as "Bullous variant of small vessel vasculitis") is a cutaneous condition in which patients with small vessel vasculitis will develop superimposed vesicles and bullae, especially on the distal extremities.Retinal vasculitisPolyarteritis nodosaLeukotriene receptor antagonist-associated Churg–Strauss syndromeCutis marmorata telangiectatica congenitaRheumatoid vasculitisImmunofluorescenceCryoglobulinemic vasculitis: Cryoglobulinemic vasculitis is a skin condition presenting in roughly 15% of people with a circulating cryoprecipitable protein.Kawasaki (surname): Kawasaki is a surname of Japanese origin (most commonly etc.) .GlomerulonephritisGiant-cell arteritisHaemophilus influenzae biogroup aegyptius: Haemophilus influenzae biogroup aegyptius (Hae) is a causative agent of acute and often purulent conjunctivitis, more commonly known as pink eye. It was discovered independently by Koch and Weeks in the 1880s.Takayasu's arteritisAutoimmune diseaseImmunosuppressive drug: Immunosuppressive drugs or immunosuppressive agents or antirejection medications are drugs that inhibit or prevent activity of the immune system. They are used in immunosuppressive therapy to:Autoantibody: An autoantibody is an antibody (a type of protein) produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases, (notably lupus erythematosus), are caused by such autoantibodies.Majocchi's disease: Majocchi's disease (also known as "Purpura annularis telangiectodes," and "Purpura annularis telangiectodes of Majocchi") is a skin condition characterized by bluish-red 1- to 3-cm annular patches composed of dark red telangiectases with petechiae.PlasmapheresisUrticariaPrednisoloneBrain biopsySystemic lupus erythematosus and pregnancy: For women with systemic lupus erythematosus (SLE), pregnancy can present some particular challenges for both mother and child.Raji cell: Raji cell line is the first continuous human cell line from hematopoietic origin. The cell lines produce an unusual strain of Epstein-Barr virus which will both transform cord blood lymphocytes and induce early antigens in Raji cells.Skip lesion: A skip lesion is a wound or inflammation that is clearly patchy, "skipping" areas that thereby are unharmed. It is a typical form of intestinal damage in Crohn's disease, but may also be the kind of damage to the renal tubules in acute tubular necrosis.Epithelial basement membrane dystrophy: Epithelial basement membrane dystrophy (EBMD), also known as map-dot-fingerprint dystrophy and Cogans's microcystic dystrophy, is a disorder of the eye that can cause pain and dryness.MethylprednisoloneCryosupernatant: The term cryosupernatant (also called cryo-poor plasma, cryoprecipitate depleted) refers to plasma from which the cryoprecipitate has been removed.Prolyl endopeptidase: Prolyl endopeptidase (PE) also known as prolyl oligopeptidase or post-proline cleaving enzyme is an enzyme that in humans is encoded by the PREP gene.ACR score for rheumatoid arthritis: ACR score is a scale to measure change in rheumatoid arthritis symptoms. It is named after the American College of Rheumatology.Autologous immune enhancement therapy: Autologous immune enhancement therapy (AIET) is a treatment method in which immune cells are taken out from the patient's body which are cultured and processed to activate them until their resistance to cancer is strengthened and then the cells are put back in the body. The cells, antibodies, and organs of the immune system work to protect and defend the body against not only tumor cells but also bacteria or viruses.Rheumatoid factor: Rheumatoid factor (RF) is the autoantibody (antibody directed against an organism's own tissues) that was first found in rheumatoid arthritis. It is defined as an antibody against the Fc portion of IgG (an antibody against an antibody).AzathioprineEggs and Marrowbone: "Eggs and Marrowbone" (Laws Q2, Roud183]) is a traditional folk song of unknown origins and multiple variations. The most well known variations are "The Old Woman From Boston" and "The Rich Old Lady".Lennox–Gastaut syndromeNeutrophil granulocyteVincristineCD4 immunoadhesin: CD4 immunoadhesin is a recombinant fusion protein consisting of a combination of CD4 and the fragment crystallizable region.Biological response modifiers: Biological response modifiers (BRMs) are substances that modify immune responses. They can be both endogenous (produced naturally within the body) and exogenous (as pharmaceutical drugs), and they can either enhance an immune response or suppress it.ScleritisCoagulative necrosis: Coagulative necrosis is a type of accidental cell death typically caused by ischemia or infarction. In coagulative necrosis the architecture of dead tissue is preserved for at least a couple of days.Gross examination

(1/1603) T lymphocyte adhesion mechanisms within inflamed human kidney: studies with a Stamper-Woodruff assay.

Renal inflammatory conditions are characterized by mononuclear cell recruitment to sites of inflammation. We have developed a modified Stamper-Woodruff assay system to analyze mechanisms of functional T cell adhesion to cryostat sections of renal biopsy material from patients with vasculitic glomerulonephritis (GN) and acute allograft rejection. Peripheral blood T cells adhered to intraglomerular, periglomerular, and tubulointerstitial regions of the cortex. Blocking monoclonal antibodies against tissue expressed ICAM-1, VCAM-1, and the CS-1 domain of fibronectin (CS-1Fn) differentially attenuated T cell adhesion. Glomerular adhesion in vasculitic GN and tubulointerstitial adhesion in acute rejection were particularly sensitive to both anti-ICAM-1 and anti-VCAM-1 antibodies, indicating a prominent role for ICAM-1 and VCAM-1 at glomerular sites in vasculitis and at tubulointerstitial sites in rejection. Furthermore, using KL/4 cells (LFA-1 expressing) and Jurkat cells (VLA-4 expressing), we demonstrated specific LFA-1/ICAM-1- and VLA-4/VCAM-1-mediated interactions within glomerular and tubulointerstitial compartments. Jurkat cells also adhered to VCAM-1-free sites, and binding was inhibitable by anti-CS-1Fn antibody, thereby demonstrating a role for VLA-4/fibronectin interactions especially at intraglomerular sites in acute rejection where VCAM-1 is notably absent. We therefore propose a prominent functional role for ICAM-1, VCAM-1, and CS-1 domain fibronectin in T cell recruitment to the inflamed kidney.  (+info)

(2/1603) The inhibition of myeloperoxidase by ceruloplasmin can be reversed by anti-myeloperoxidase antibodies.

BACKGROUND: The purpose of this study was to characterize the recently reported inhibition of myeloperoxidase (MPO) by ceruloplasmin and to determine whether this may be disturbed in the presence of anti-MPO antibodies. METHODS: Specificity of the binding between ceruloplasmin and MPO was confirmed by Western blotting and enzyme-linked immunosorbent assay (ELISA), and the enzymatic activity of MPO was measured in the presence of ceruloplasmin, affinity-purified anti-MPO antibodies, or both. The affinity of the binding between MPO and ceruloplasmin and MPO and the anti-MPO antibodies was measured using a biosensor, with the results confirmed by chaotrope ELISA. RESULTS: Affinity-purified anti-MPO antibodies from patients with microscopic polyangiitis and florid renal vasculitis inhibited the binding between ceruloplasmin and MPO to a maximum of 72.9 +/- 12.8%, whereas those from patients with Wegener's granulomatosis and only minimal renal involvement inhibited the binding to a maximum of only 36.8 +/- 10.9% (P < 0. 001), with comparable reversal of the ceruloplasmin-mediated inhibition of MPO activity. Measurement of the affinity of the interactions demonstrated that binding between MPO and the anti-MPO antibodies is stronger than that between MPO and ceruloplasmin (1.61 x 107 to 1.33 x 108 vs. 7.46 x 106 m-1), indicating that binding to the autoantibody would be favored in vivo. CONCLUSIONS: This study confirms a role for ceruloplasmin as a physiological inhibitor of MPO, and demonstrates how the inhibition may be disrupted in the presence of anti-MPO antibodies. Because a majority (16 of 21) of the antibodies did not themselves inhibit MPO activity, their interference with the inhibition mediated by ceruloplasmin may be brought about by steric hindrance consequent upon the binding of the antibody to a dominant epitope at or near the active site.  (+info)

(3/1603) Systemic candidiasis with candida vasculitis due to Candida kruzei in a patient with acute myeloid leukaemia.

Candida kruzei-related systemic infections are increasing in frequency, particularly in patients receiving prophylaxis with antifungal triazoles. A Caucasian male with newly diagnosed acute myeloid leukaemia (AML M1) developed severe and persistent fever associated with a micropustular eruption scattered over the trunk and limbs during induction chemotherapy. Blood cultures grew Candida kruzei, and biopsies of the skin lesions revealed a candida vasculitis. He responded to high doses of liposomal amphotericin B and was discharged well from hospital.  (+info)

(4/1603) Kawasaki syndrome.

KS is a fascinating illness of childhood that has emerged over the last 30 years and is now recognized as the most common cause of acquired heart disease in children in the United States. It has a dramatic clinical presentation in most cases, but for a small number of cases that present with a more subtle disease there appears to be a greater risk of coronary artery complications. Progress has been made in the management of KS, but although the disease appears to be related to an infectious etiology, no infectious agent has been proven to be the cause. Finally, issues in long-term management and prognosis are yet to be clarified.  (+info)

(5/1603) Vasculitis in the Palmerston North mouse model of lupus: phenotype and cytokine production profile of infiltrating cells.

OBJECTIVE: To define the phenotype of cells in the perivascular and vascular infiltrates of Palmerston North (PN) mice and the cytokines that those cells produce. METHODS: Immunohistologic analysis, flow cytometric analysis, and reverse transcriptase-polymerase chain reaction (RT-PCR) studies were performed on tissues and cells from female PN mice and age-matched and sex-matched DBA/2 mice. RESULTS: With aging, PN mice developed a female-predominant, lupus-like disease, with a severe systemic mononuclear cell perivasculitis and vasculitis. The perivasculitis involved arteries and veins in kidney, liver, brain, and lung; the vasculitis predominantly involved veins and venules. The perivascular and vascular infiltrates in female PN mice were composed mainly of an unusual cell type that expressed phenotypic markers characteristic of both T cells (Thy1+, CD3+, CD4+, T cell receptor + [TCR+]) and B cells (B220+). In addition, the infiltrates contained a smaller number of conventional CD4+,B220- T cells and macrophages. Very few CD8+ T cells or surface Ig+ B cells were seen. Unlike the Thy1+,B220+ T cells present in MRL/lpr mice, most of which were CD4-,CD8- and TCRalpha/beta+, the majority of the Thy1+,B220+ T cells in the perivascular/vascular infiltrates of PN mice were CD4+ and expressed either TCRalpha/beta or TCRgamma/delta. By immunohistologic staining, the cells in the perivascular and vascular infiltrates in the kidneys of older PN mice were shown to produce interleukin-4 (IL-4), IL-6, and IL-10, but not IL-2, interferon-gamma, transforming growth factor beta, tumor necrosis factor alpha, or IL-1beta. By RT-PCR, the kidneys of older PN mice were found to express high levels of IL-4, IL-6, and IL-10 messenger RNA. CONCLUSION: The vascular and perivascular infiltrates in PN mice are composed predominantly of an unusual subpopulation of T cells that are Thy1+,B220+,CD4+,CD8-, express either TCRalpha/beta or TCRgamma/delta, and produce mainly type 2 cytokines. The exact role of these cells in the immunopathogenesis of lupus-like disease in PN mice remains to be elucidated.  (+info)

(6/1603) Anti-endothelial cell antibodies in systemic vasculitis and systemic lupus erythematosus (SLE): effects of heat inactivation on binding and specificity.

Heating sera is used to inactivate complement but may affect the binding characteristics of autoantibodies. We studied the effect of heating sera from patients with systemic vasculitides and SLE on antibody binding to cultured human umbilical vein endothelial cells. Sera from 32 patients with systemic vasculitides, eight with SLE and 10 healthy controls were studied for anti-endothelial cell antibodies (AECA) using an ELISA before and after heating sera to 56 degrees C for 30 min. The median (range) AECA binding index in the patient group increased from 20% (0-153%) to 71.5% (10-259%) (P < 0.0001). The AECA binding index in the control group also increased from 14% (0-52%) to 90% (42-154%) (P < 0.0001). The increased binding was unaffected by the addition of fresh complement or removal of immune complexes and the increased binding after heating persisted even after cooling to 4 degrees C. Specificity experiments showed that after heating, the binding specificity of sera was lost. Removal of immunoglobulin with Protein A abolished the increased binding seen after heating. Heating sera increases AECA binding in both patient and control sera. The mechanism is probably non-specific damage to the immunoglobulin molecule, and heating sera should thus be avoided.  (+info)

(7/1603) Aldose reductase functions as a detoxification system for lipid peroxidation products in vasculitis.

Giant cell arteritis (GCA) is a systemic vasculitis preferentially affecting large and medium-sized arteries. Inflammatory infiltrates in the arterial wall induce luminal occlusion with subsequent ischemia and degradation of the elastic membranes, allowing aneurysm formation. To identify pathways relevant to the disease process, differential display-PCR was used. The enzyme aldose reductase (AR), which is implicated in the regulation of tissue osmolarity, was found to be upregulated in the arteritic lesions. Upregulated AR expression was limited to areas of tissue destruction in inflamed arteries, where it was detected in T cells, macrophages, and smooth muscle cells. The production of AR was highly correlated with the presence of 4-hydroxynonenal (HNE), a toxic aldehyde and downstream product of lipid peroxidation. In vitro exposure of mononuclear cells to HNE was sufficient to induce AR production. The in vivo relationship of AR and HNE was explored by treating human GCA temporal artery-severe combined immunodeficiency (SCID) mouse chimeras with the AR inhibitors Sorbinil and Zopolrestat. Inhibition of AR increased HNE adducts twofold and the number of apoptotic cells in the arterial wall threefold. These data demonstrate that AR has a tissue-protective function by preventing damage from lipid peroxidation. We propose that AR is an oxidative defense mechanism able to neutralize the toxic effects of lipid peroxidation and has a role in limiting the arterial wall injury mediated by reactive oxygen species.  (+info)

(8/1603) Adenovirus-mediated delivery of fas ligand inhibits intimal hyperplasia after balloon injury in immunologically primed animals.

BACKGROUND: Adenoviral constructs have been used for studies of injury-induced vascular hyperplasia in immunologically naive laboratory animals, but their usefulness for intra-arterial gene therapy may be limited by the prevalence of preexisting immunity to adenovirus in the patient population. Here, we explored the efficacy of adenovirus-mediated transfer of Fas ligand, a cytotoxic gene with immunomodulatory properties, in inhibiting injury-induced vascular lesion formation in both naive and immunologically primed animals. METHODS AND RESULTS: Lesion formation was evaluated in balloon-injured carotid arteries of naive and adenovirus-immunized rats that were infected with adenoviral constructs expressing Fas ligand (Ad-FasL), the cyclin-dependent kinase inhibitor p21 (Ad-p21), or beta-galactosidase (Ad-betagal). In naive rats, Ad-FasL induced apoptosis in medial vascular smooth muscle cells and inhibited intimal hyperplasia by 60% relative to Ad-betagal-treated vessels (P<0.05), whereas the cytostatic agent Ad-p21 decreased lesion size by 58% (P<0.05). In animals preimmunized with an adenoviral vector containing no transgene, Ad-FasL significantly inhibited neointima formation (73% reduction, P<0.05), but Ad-p21 failed to inhibit neointima formation relative to controls. Immunologically primed rats displayed robust T-cell infiltration in Ad-p21- and Ad-betagal-treated vessels, but T-cell infiltration was markedly attenuated in Ad-FasL-treated vessels. CONCLUSIONS: Our data demonstrate that adenovirus-mediated Fas ligand delivery can inhibit intimal hyperplasia in both immunologically primed and naive animals, whereas the efficacy of an adenovirus-mediated p21 delivery is limited to immunologically naive animals. This study documents, for the first time, the therapeutic efficacy of intravascular adenoviral gene transfer in animals with preexisting immunity to adenovirus.  (+info)


  • Polyarteritis nodosa is a form of necrotizing vasculitis of the small and medium sized muscular arteries, renal arteries and visceral arteries which may spread to surrounding veins. (
  • Cutaneous polyarteritis nodosa is the variant of polyarteritis nodosa which produce symptomatic skin vasculitis symptoms and signs. (
  • Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects medium-sized muscular arteries, with occasional involvement of small muscular arteries [ 1,2 ]. (
  • Unlike some other vasculitides (eg, microscopic polyarteritis, granulomatosis with polyangiitis [Wegener's]), polyarteritis nodosa is not associated with antineutrophil cytoplasmic antibodies (ANCA) [ 3 ]. (
  • Types of vasculitis: polyarteritis nodosa. (


  • Microscopic polyangiitis and other ANCA-associated systemic vasculitides (eg, granulomatosis with polyangiitis [Wegener's] and eosinophilic granulomatosis with polyangiitis [Churg-Strauss]) that characteristically affect small vessels such as arterioles, capillaries, and venules, as well as muscular arteries, are discussed separately. (
  • Giant cell arteritis is a vasculitis that affects large and medium-size arteries. (
  • This is a medium vessel vasculitis that affects many arteries. (
  • And so, if we break it down, we can see poly means many, arter refers to the arteries, and itis is inflammation, just like any vasculitis you see some inflammation, and nodosa means nodular. (


  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (ANCA Vasculitis) market report helps in identifying prominent locations for conducting clinical trials which saves time and cost. (
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (ANCA Vasculitis) market report offers coverage of disease clinical trials by region, country (G7 & E7), phase, trial status, end points status and sponsor type. (
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (ANCA Vasculitis) Global Clinical Trials Review, H1, 2017" provides an overview of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (ANCA Vasculitis) clinical trials scenario. (
  • This report provides top line data relating to the clinical trials on Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (ANCA Vasculitis). (
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (ANCA Vasculitis) market report also provides prominent drugs for in-progress trials (based on number of ongoing trials). (
  • Most importantly, the establishment and acceptance of microscopic polyangiitis as a distinct disease, and the routine availability and recognition of ANCA testing as critical to diagnosing vasculitides other than PAN, have led to more patients with vasculitis being classified as not having PAN. (


  • This study examines macular blood flow using OCT angiography in eyes with angiographically active retinal vasculitis compared to normal eyes. (
  • Adult patient with retinal vasculitis were imaged with fluorescein angiography (FA) and OCT angiography with 70 kHz OCT, using the split-spectrum amplitude decorrelation angiography algorithm (SSADA). (
  • 5 patients (7 eyes) with angiographically active retinal vasculitis were included in the study. (
  • Their diagnoses included lupus retinal vasculitis with choroiditis, Bechet's disease, TINU with retinal vasculits, sarcoidosis, and idiopathic retinal vasculitis. (
  • In eyes with retinal vasculitis, the average parafoveal vessel density (see Figure 1B for example) was significantly lower, at 79.8% (95% CI 76.3-83.4, p=0.006). (
  • Patients with retinal vasculitis have significantly lower parafoveal vessel density compared to normal eyes, as measured by OCT angiography. (
  • This technique shows promise as a possible biomarker for determining disease activity, and gauging treatment response in patients with retinal vasculitis. (
  • Fig1B: OCT angiography of a patient with lupus retinal vasculitis, displaying lower parafoveal vascular density. (



  • Patients with noninfectious aortitis related to large-vessel vasculitis may present with symptoms of arterial insufficiency in the case of Takayasu arteritis or a characteristic headache with giant cell arteritis. (
  • Lower parafoveal vessel density was noted even in patients who had only peripheral vasculitis on FA. (
  • So, remember with vasculitis the body is mistaking the blood vessels to be a foreign protein or something foreign, and so antibodies, little Y-shaped proteins that are released by the immune system, are targeting the blood vessel wall, because they think it's foreign. (


  • Patient cohorts of PAN included in case series and other research studies published prior to the 1990s almost certainly included a mixture of the newer definition of PAN with microscopic polyangiitis and possibly other forms of vasculitis. (
  • We also imaged choroidal blood flow in the patient with lupus vasculitis and choroiditis. (