VasculitisEosinophilicWegener's granulomatosis and microscopic polyangiitisGlomerulonephritisAntineutrophil cytoplasmicVasculitidesPolyarteritisANCAFormerly knownRheumatoidRemissionCutaneousPatientsClinicalAutoantibodiesDiagnosisWegener GranulomatosisInductionDiseaseCyclophosphamideTherapeutic approachesBlood vesselsRespiratoryAffectsPolymorphismCohortSymptomsIncludeTrialsSyndromeSmall
Vasculitis13
- INTRODUCTION - "Classic" granulomatosis with polyangiitis (GPA) is a form of systemic vasculitis (polyangiitis) with necrotizing granulomatous inflammation of the upper and lower respiratory tracts, systemic necrotizing vasculitis, and necrotizing glomerulonephritis [ 1,2 ]. (medilib.ir)
- Microscopic polyangiitis (MPA) is a necrotizing vasculitis without granulomatous inflammation that predominantly affects small vessels (ie, capillaries, venules, or arterioles) and can present with pulmonary capillaritis or in the context of interstitial lung disease [ 3 ]. (medilib.ir)
- Henoch-Schönlein purpura, granulomatosis with polyangiitis or GPA, eosinophilic granulomatosis with polyangiitis or EGPA, previously referred to as Churg-Strauss syndrome), cutaneous leukocytoclastic vasculitis, microscopic polyangiitis). (printo.it)
- The incidence and clinical features of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) have been shown to vary according to geographical areas, with granulomatosis with polyangiitis (GPA) being more common in northern Europe and microscopic polyangiitis (MPA) being more common in Asian countries. (nih.gov)
- Churg-Strauss Syndrome (CSS), now also referred to by its medically more accurate term eosinophilic granulomatosis with polyangiitis (EGPA), is a rare systemic vasculitis (inflammation in the wall of blood vessels of the body), predominantly affecting small-sized vessels. (vasculitisfoundation.org)
- Dr. Jennette's more basic research (funded by NIH NIDDK) focuses on the pathogenesis of glomerulonephritis, vasculitis and granulomatosis caused by anti-neutrophil cytoplasmic autoantibodies (ANCA) using in vitro assays, animal models, and human specimens. (unc.edu)
- Wegener's granulomatosis is a form of vasculitis that affects the lungs, kidneys and other organs. (checkorphan.org)
- Objectives Because standard immunosuppressive treatment for antineutrophil cytoplasm antibody-associated vasculitis (AAV) (granulomatosis with polyangiitis (Wegener's) (GPA) and microscopic polyangiitis (MPA)) has been associated with a significant risk of developing cancer, the cancer incidence of treated AAV patients was assessed. (bmj.com)
- To determine the reliability of self-reported diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) from a large, international, internet-based cohort. (vasculitisfoundation.org)
- Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of diseases (granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis), characterized by destruction and inflammation of small vessels. (nih.gov)
- Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, small- and medium-sized vessel vasculitis, and focal necrotizing glomerulonephritis, often with crescent formation. (merckmanuals.com)
- Although Henoch-Schonlein purpura (HSP) and Kawasaki disease (KD) are quite common forms of vasculitis, polyarteritis nodosa (PAN), Wegener's granulomatosis (WG) and Takayasu arteritis (TA) are diagnosed less commonly in children. (cyberleninka.org)
- To provide evidence-based recommendations and expert guidance for the management of antineutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). (bvsalud.org)
Eosinophilic9
- females Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). (wikipedia.org)
- Eosinophilic granulomatosis with polyangiitis (EGPA). (medlineplus.gov)
- AAV include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). (smw.ch)
- AAV is a group of three diseases including eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, and microscopic polyangiitis. (openpr.com)
- Eosinophilic granulomatosis with polyangiitis (EGPA) is part of antineutrophil cytoplasmic antibodies (ANCAs)-associated vasculitides. (biomedcentral.com)
- We report a single-center cohort of patients with eosinophilic granulomatosis with polyangiitis. (biomedcentral.com)
- The spectrum of AAV comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), the later formerly known as Churg-Strauss syndrome (CSS). (biomedcentral.com)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) - affects cells in the blood vessels of the lungs, gastrointestinal track, skin, and nerves. (upmc.com)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): evolutions in classification, etiopathogenesis, assessment and management. (medscape.com)
Wegener's granulomatosis and microscopic polyangiitis4
- Granulomatosis with Polyangiitis (GPA) (Wegener's Granulomatosis) and Microscopic Polyangiitis (MPA) in adult patients in combination with glucocorticoids ( 1.4 ). (drugs.com)
- A model to predict cardiovascular events in patients with newly diagnosed wegener's granulomatosis and microscopic polyangiitis. (ox.ac.uk)
- The primary systemic vasculitides usually associated with autoantibodies to neutrophil cytoplasmic antigens include Wegener's granulomatosis and microscopic polyangiitis. (nih.gov)
- Many trials include data on pooled groups of people with wegener's granulomatosis and microscopic polyangiitis. (checkorphan.org)
Glomerulonephritis1
- Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. (smw.ch)
Antineutrophil cytoplasmic4
- Almost all patients with granulomatosis with polyangiitis have c-antineutrophil cytoplasmic antibody, but not vice versa. (wikipedia.org)
- Thought to be part of a group that includes granulomatosis with polyangiitis since both are associated with antineutrophil cytoplasmic antibody and similar extrapulmonary manifestations. (wikipedia.org)
- Because antineutrophil cytoplasmic auto-antibodies (ANCA) can be detected in the serum (liquid and a cellular part of the blood) in up to 40% of the patients, it is also considered one of the three ANCA-associated vasculitides, along with granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis. (vasculitisfoundation.org)
- Wegener's granulomatosis is part of a larger group of vasculitic syndromes, all of which feature the presence of an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) and affect small and medium-size blood vessels. (checkorphan.org)
Vasculitides3
- Objectives Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are autoimmune vasculitides associated with antineutrophil cytoplasm antibodies that target proteinase 3 (PR3) or myeloperoxidase (MPO) found within neutrophils and monocytes. (bmj.com)
- Although Wegener's granulomatosis affects small and medium-sized vessels, it is formally classified as one of the small vessel vasculitides in the Chapel Hill system. (checkorphan.org)
- Protective Role of HLA-DRB1*13:02 against Microscopic Polyangiitis and MPO-ANCA-Positive Vasculitides in a Japanese Population: A Case-Control Study. (cdc.gov)
Polyarteritis1
- 10% on complete blood count Mononeuropathy or polyneuropathy Migratory or transient pulmonary opacities on chest x-ray Paranasal sinus abnormalities Vessel biopsy showing eosinophils in extravascular areas Microscopic polyarteritis/polyangiitis. (wikipedia.org)
ANCA1
- [ 2 ] Subsequently, ANCA positivity was found to correlate closely with the clinical syndromes of Wegener granulomatosis, Churg-Strauss syndrome, and microscopic polyangiitis. (medscape.com)
Formerly known2
- Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis) is a multisystem disease characterized by a disseminated necrotizing inflammation involving the small blood vessels and the surrounding tissue. (medscape.com)
- Granulomatosis with polyangiitis (GPA) (formerly known as Wegener's granulomatosis) is an uncommon disease affecting the sinuses, nose and lungs and frequently involves the kidneys, lungs, eyes, ears, throat, skin and other body organs. (vasculitisfoundation.org)
Rheumatoid1
- ABP 798 is being developed as a biosimilar candidate to Rituxan, an anti-CD20 monoclonal antibody that is approved in many regions for the treatment of adult patients with non-Hodgkin's lymphoma, chronic lymphocytic leukemia, moderate-to-severe rheumatoid arthritis, pemphigus vulgaris, granulomatosis with polyangiitis and microscopic polyangiitis. (amgen.com)
Remission2
- Szpirt WM, Heaf JG, Petersen J. Plasma exchange for induction and cyclosporine A for maintenance of remission in Wegener's granulomatosis - a clinical randomized controlled trial. (smw.ch)
- The standard treatment for Wegener's granulomatosis is cyclophosphamide and high dose corticosteroids for remission induction and less toxic immunosuppressants like azathioprine, leflunomide, methotrexate or mycophenolate mofetil. (checkorphan.org)
Cutaneous1
- Disorders to be considered in the differential diagnosis of cutaneous granulomatosis with polyangiitis lesions are listed below. (medscape.com)
Patients6
- CLINICAL FEATURES - Patients with granulomatosis with polyangiitis (GPA) typically have both upper airway and pulmonary involvement, although a minority may have just one or the other [ 1 ]. (medilib.ir)
- To create a prognostic tool to quantify the 5 year cardiovascular (CV) risk in patients with newly diagnosed Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) without pre-morbid CV disease. (ox.ac.uk)
- Although the structure of the granulomas and multinucleated giant cells in granulomatosis with polyangiitis (GPA) is known, little is established about what drove granuloma formation and why it is infrequently found in patients with myeloperoxidase-antineutrophil cytoplasm antibodies. (bmj.com)
- Wegener's granulomatosis: studies in eighteen patients and a review of the literature. (smw.ch)
- Wegener granulomatosis: an analysis of 158 patients. (smw.ch)
- The relapse rate was lower among the patients with microscopic polyangiitis than among those with Wegener's granulomatosis (P=0.03). (nih.gov)
Clinical2
- See "Granulomatosis with polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis" . (medilib.ir)
- Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment. (medscape.com)
Autoantibodies1
- Positive serological tests for these autoantibodies support the suspicion for granulomatosis with polyangiitis. (medscape.com)
Diagnosis1
- Histopathological examination is required to make a reliable diagnosis of granulomatosis with polyangiitis. (medscape.com)
Wegener Granulomatosis6
- examples include Wegener granulomatosis and microscopic polyangiitis. (medscape.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)
- Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation. (medscape.com)
- Sinonasal Wegener granulomatosis: a single-institution experience with 120 cases. (medscape.com)
- [ 1 ] A retrospective review of 34 cases of DAH revealed nearly one-third of the cases were caused by Wegener granulomatosis. (medscape.com)
Induction1
- See "Granulomatosis with polyangiitis and microscopic polyangiitis: Induction and maintenance therapy" . (medilib.ir)
Disease1
- The patient had a history of granulomatosis with polyangiitis, chronic sinusitis, chronic kidney disease, and pulmonary coccidioidomycosis. (cdc.gov)
Cyclophosphamide1
- Novack SN, Pearson CM. Cyclophosphamide therapy in Wegener's granulomatosis. (smw.ch)
Therapeutic approaches1
- Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches. (medscape.com)
Blood vessels1
- Microscopic polyangiitis - affects cells in the blood vessels in organs throughout the whole body. (upmc.com)
Respiratory2
- Microscopic polyangiitis (MPA) affecting the respiratory tract is most often associated with alveolar hemorrhage [ 6,7 ]. (medilib.ir)
- Despite the new immunosuppressive and immunomo-dulatory therapies, WG, microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS) still carry considerable morbidity and mortality mainly due to progressive renal failure or aggressive respiratory involvement. (cyberleninka.org)
Affects1
- Affects medium and small vessels with vascular and extravascular granulomatosis. (wikipedia.org)
Polymorphism1
- Contrasting association of a non-synonymous leptin receptor gene polymorphism with Wegener's granulomatosis and Churg-Strauss syndrome. (cdc.gov)
Cohort1
- The model was tested using the Wegener's Granulomatosis Etanercept Trial (WGET) cohort. (ox.ac.uk)
Symptoms1
- Wegener's granulomatosis is usually only suspected when a patient has had unexplained symptoms for a long period of time. (checkorphan.org)
Include1
- Examples include giant cell arteritis, microscopic polyangiitis, and granulomatosis with polyangiitis. (wikipedia.org)
Trials1
- A systematic review of 84 trials examined the evidence for various treatments in Wegener's granulomatosis. (checkorphan.org)
Syndrome1
- Apart from Wegener's, this category includes Churg-Strauss syndrome and microscopic polyangiitis. (checkorphan.org)
Small1
- If only small vessels are affected, it is called microscopic polyangiitis, although it is more associated with granulomatosis with polyangiitis than to classic PAN. (wikipedia.org)