• 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)
  • AAV include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). (smw.ch)
  • We are pleased to announce a decision to fund mepolizumab (Nucala) from 1 April 2020 for the treatment of severe refractory eosinophilic asthma, through an agreement with GlaxoSmithKline NZ Limited (GSK). (pharmac.govt.nz)
  • Eosinophilic granulomatosis with polyangiitis is a systemic small- and medium-vessel necrotizing vasculitis, characterized by extravascular granulomas, eosinophilia, and tissue infiltration by eosinophils. (msdmanuals.com)
  • Eosinophilic granulomatosis with polyangiitis (EGPA) occurs in about 3 people/million. (msdmanuals.com)
  • The cause of eosinophilic granulomatosis with polyangiitis is unknown. (msdmanuals.com)
  • Rituximab as Induction Therapy in Eosinophilic Granulomatosis with Polyangiitis Refractory to Conventional Immunosuppressive Treatment: A 36-Month Follow-Up Analysis. (research.com)
  • BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is often associated with glucocorticoid-dependent asthma and/or ear, nose and throat (ENT) manifestations. (bvsalud.org)
  • Positive high-level results from the MANDARA Phase III trial showed AstraZeneca's FASENRA (benralizumab) met the primary endpoint of the trial and demonstrated non-inferior rates of remission compared to mepolizumab in patients with eosinophilic granulomatosis with polyangiitis (EGPA) who were receiving oral corticosteroids (OCS) with or without stable immunosuppressive therapy. (kanerepublican.com)
  • Dr Michael Wechsler, Principal Investigator said: "The positive MANDARA trial results are exciting because patients with eosinophilic granulomatosis with polyangiitis today have limited treatment options but face crippling symptoms, which can even be fatal if not treated. (kanerepublican.com)
  • This trial demonstrates that a biologic medicine given in a single monthly injection could help patients achieve remission rates comparable to the current standard of care, adding to the importance of benralizumab as a potential treatment option for eosinophilic granulomatosis with polyangiitis. (kanerepublican.com)
  • Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss vasculitis). (clevelandclinic.org)
  • Differential diagnoses include drug allergies and parasitic infections, solid and hematological malignancies (i.e. chronic myeloid leukemia), eosinophilic granulomatosis with polyangiitis and human T cell lymphotropic virus infection (see these terms). (orpha.net)
  • The study is open to adults diagnosed with certain types of vasculitis, called Granulomatosis Polyangiitis (GPA), Microscopic Polyangiitis (MPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA). (cardiff.ac.uk)
  • Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic antibody-associated vasculitis typically characterized by asthma, peripheral eosinophilia and medium- to small-vessel necrotizing vasculitis. (the-rheumatologist.org)
  • A Case: Churg-Strauss With Cardiac Involvement A patient case demonstrates the somewhat common cardiac involvement often seen in eosinophilic granulomatosis with polyangiitis. (medscape.com)
  • We describe the safety and efficacy of off-label use of dupilumab in relapsing and/or refractory EGPA. (bvsalud.org)
  • Brief Summary This is a randomized, double blind, active-controlled, parallel group, multicenter 52-week Phase 3 study to compare the efficacy and safety of benralizumab 30 mg versus mepolizumab 300 mg administered by subcutaneous (SC) injection in patients with relapsing or refractory EGPA on corticosteroid therapy with or without stable immunosuppressive therapy. (cardiff.ac.uk)
  • Objective This study was conducted to evaluate the efficacy and safety of repeated and prolonged B cell depletion with rituximab (RTX) for the maintenance of long-term remission in patients with chronic relapsing granulomatosis with polyangiitis (Wegener's) (GPA). (elsevierpure.com)
  • Wegener's granulomatosis. (smw.ch)
  • Wegener's granulomatosis: studies in eighteen patients and a review of the literature. (smw.ch)
  • Novack SN, Pearson CM. Cyclophosphamide therapy in Wegener's granulomatosis. (smw.ch)
  • Lamprecht P, Gross WL, Kabelitz D. T cell alterations and lymphoid neogenesis favoring autoimmunity in Wegener's granulomatosis. (smw.ch)
  • Differential B- and T-cell activation in Wegener's granulomatosis. (smw.ch)
  • 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)
  • Clinical features and outcome of pediatric Wegener's granulomatosis. (medscape.com)
  • Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. (medscape.com)
  • Two cases of refractory Wegener's granulomatosis successfully treated with rituximab. (edu.pl)
  • Granulomatosis with polyangiitis (GPA, previously called Wegener's granulomatosis) is a rare, systemic, antineutrophil cytoplasmic antibody (ANCA)-associated form of vasculitis whose lesions usually affect the respiratory tract and kidneys [ 1 ]. (ejao.org)
  • Renal histopathology and clinical course in 94 patients with Wegener's granulomatosis. (mediar-press.net)
  • A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis. (mediar-press.net)
  • Objective Treatment-refractory antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a life-threatening condition without evidence-based treatment options. (bmj.com)
  • Granulomatosis with polyangiitis (GPA) is a chronic and systematic autoimmune condition characterized by granuloma formation and necrotizing vasculitis of small to medium-sized vessels. (ejao.org)
  • Effective and safe treatment of anti-CD38 therapy in systemic lupus erythematosus-associated refractory cerebral vasculitis induces immune tolerance. (uk-erlangen.de)
  • Treatment of refractory hypocomplementaemic urticarial vasculitis syndrome progressing to systemic lupus erythematosus with belimumab. (uk-erlangen.de)
  • Scleritis may be isolated to the eye but is commonly associated with systemic autoimmune disorders, including rheumatoid arthritis , systemic lupus erythematosus , relapsing polychondritis , spondyloarthropathies , Wegener granulomatosis , polyarteritis nodosa , and giant cell arteritis . (medscape.com)
  • A 25-year-old Mexican American woman with a five-year history of systemic lupus erythematosus (SLE) presents with refractory, acute cutaneous lupus erythematosus (ACLE) and subacute cutaneous lupus erythematosus (SCLE) affecting the scalp, face and hands. (the-rheumatologist.org)
  • Wegener granulomatosis: an analysis of 158 patients. (smw.ch)
  • Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. (medscape.com)
  • Manifestations are similar to granulomatosis with polyangiitis . (amboss.com)
  • There are few case reports describing the successful use of Rituximab in refractory sarcoidosis with lung, eye, lymph nodes and skin involvement. (biomedcentral.com)
  • In this paper we described three different case reports in which Rituximab has been used to treat refractory sarcoidosis and we reviewed the existing literature. (biomedcentral.com)
  • A prospective phase I/II trial has also been published investigating the role of rituximab in the treatment of refractory pulmonary sarcoidosis [ 16 ]. (biomedcentral.com)
  • Herein we describe the impact of Rituximab in three diverse cases of refractory sarcoidosis with different organs involvement and we review the currently available evidence regarding the use of the anti-CD20 monoclonal antibody as a therapeutic option in this granulomatous disease. (biomedcentral.com)
  • The second patient with proteinase 3-ANCA-positive granulomatosis with polyangiitis, diffuse alveolar haemorrhage necessitating extracorporeal membrane oxygenation (ECMO) and acute kidney failure, requiring kidney replacement therapy, was weaned off ECMO, mechanical ventilation and dialysis and discharged home after daratumumab. (bmj.com)
  • Cervical Spine Fusion for the Treatment of Refractory Idiopathic Cervical Dystonia Case study illustrating the multifaceted approach necessary to treat refractory cervical dystonia with concomitant rigid cervical kyphosis, in the face of neuromuscular pathology. (medscape.com)
  • Of these patients, nine (six ANCA-positive, three ANCA-negative) had been treated with RTX for relapsing or refractory disease on standard immunosuppressive treatment. (biomedcentral.com)
  • 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)
  • Teclistamab, in combination with daratumumab, is in clinical development for the treatment of adults with relapsed or refractory multiple myeloma (MM) who have had previous treatment. (nihr.ac.uk)
  • The mainstay of treatment for granulomatosis with polyangiitis (GPA) is a combination of corticosteroids and cytotoxic agents. (medscape.com)
  • Conclusion Daratumumab was safe and effective in inducing remission in two patients with severe treatment-refractory AAV, warranting prospective clinical trials to establish safety and efficacy. (bmj.com)
  • Most patients undergo spontaneous resolution or present a good response to systemic treatment, but a small percentage of patients develop a chronic, progressive disease, which can be refractory to multiple lines of treatment [ 1 ]. (biomedcentral.com)
  • For refractory collagen diseases that are resistant to existing therapies, we endeavor to improve clinical outcomes using state-of-the-art treatment methods such as biological products, immunosuppressive therapy, plasmapheresis, and immunoabsorption therapy. (okayama-u.ac.jp)
  • Epilepsy: refractory epilepsy requiring immune modulation treatment. (clevelandclinic.org)
  • An FDA review found maribavir to be superior to investigator-assigned treatment (IAT) (ganciclovir, valganciclovir, foscarnet, or cidofovir) in clearing CMV DNA from plasma in a population of transplant patients with refractory CMV. (prescriberight.com)
  • The FDA's Antimicrobial Drugs Advisory Committee (AMDAC) voted unanimously to recommend maribavir for the treatment of refractory cytomegalovirus (CMV) infection with or without resistance to ganciclovir, valganciclovir, foscarnet or cidofovir in transplant recipients. (prescriberight.com)
  • [3] [4] These symptoms will typically be refractory to dry eye treatment. (eyewiki.org)
  • Clinical features are very similar to those of granulomatosis with polyangiitis ( GPA ), but MPA spares the vessels in the upper respiratory tract (no sinusitis or rhinitis ). (amboss.com)
  • Sarcoidosis is a granulomatous disease whose outcome varies from spontaneous remission to chronic refractory disease. (biomedcentral.com)
  • Cases of cicatrizing conjunctivitis secondary to systemic inflammatory conditions such as Sjogren's syndrome, granulomatosis with polyangiitis (GPA) , and sarcoidosis have also been reported. (eyewiki.org)
  • Granulomatosis with polyangiitis (formerly known as Wegner's granulomatosis). (clevelandclinic.org)
  • Scleritis commonly has an underlying cause, usually an autoimmune disease (rheumatoid arthritis, granulomatosis with polyangiitis, and other vasculitic/connective tissue diseases). (medscape.com)
  • We have a history of practice ahead of other medical institutions and plenty of experience in immunosuppressive therapy, biological products, plasmapheresis, and immunoadsorption therapy for patients with collagen disease that are severe, refractory, and accompanied with complications in addition to typical cases. (okayama-u.ac.jp)
  • [20] Lower doses of azathioprine are used as a therapy in children with refractory or corticosteroid-dependent Crohn's disease, without causing many side effects. (wikizero.com)
  • Effects of casirivimab/imdevimab on systemic and mucosal immunity against SARS-CoV-2 in B-cell depleted patients with autoimmune rheumatic diseases refractory to vaccination. (uk-erlangen.de)
  • However, refractory disease is still a great challenge. (biomedcentral.com)
  • Dynamic Changes in the Nasal Microbiome Associated With Disease Activity in Patients With Granulomatosis With Polyangiitis. (upenn.edu)
  • Necrotic, purpuric, and blistering plaque on the wrist in a patient with granulomatosis with polyangiitis. (medscape.com)
  • Nguyen Y, Guillevin L. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss). (medscape.com)
  • The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). (medscape.com)
  • See "Epidemiology, pathogenesis, and pathology of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)" . (medilib.ir)
  • See "Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Treatment and prognosis" . (medilib.ir)
  • The most commonly cited causes are rheumatoid arthritis (RA), granulomatosis with polyangiitis (GPA) and various dermatoses. (avehjournal.org)
  • For severe GPA that is refractory to remisison induction with rituximab or cyclophosphamide, switch treatment to the other therapy rather than combining the 2 therapies. (medscape.com)
  • Rituximab and omalizumab are often used off-label, especially in steroid-refractory or relapsing cases. (medscape.com)
  • Starr MR, Garrity JA, Tooley AA, Salom?o DR. Clinical and histopathological findings of patients with orbital granulomatosis with polyangiitis cases refractory to rituximab. (jefferson.edu)
  • 7. Evidence for the efficacy and safety of anti-interleukin-5 treatment in the management of refractory eosinophilic asthma. (nih.gov)
  • Mepolizumab, sold under the brand name Nucala by GlaxoSmithKline, is a humanized monoclonal antibody used for the treatment of severe eosinophilic asthma, eosinophilic granulomatosis, and hypereosinophilic syndrome (HES). (alleviareindia.com)
  • 6] In the European Union it is approved as an add-on treatment for severe refractory eosinophilic asthma in adults. (alleviareindia.com)
  • Current Indications: eosinophilic asthma, eosinophilic granulomatosis, and hypereosinophilic syndrome (HES). (alleviareindia.com)
  • The mainstay of treatment for granulomatosis with polyangiitis (GPA) is a combination of corticosteroids and cytotoxic agents. (medscape.com)
  • For the treatment of primary chronic immune thrombocytopenia (ITP) in adult patients who are refractory to other treatments (e.g. corticosteroids, immunoglobulins). (ncpe.ie)
  • EPIDEMIOLOGY - The prevalence of granulomatosis with polyangiitis (GPA) ranges from 2.3 to 146.0 cases per million persons, with an incidence of 0.4 to 11.9 cases per million person-years [ 3 ]. (medilib.ir)
  • 6. The roles of IL-5 and anti-IL-5 treatment in eosinophilic diseases: Asthma, eosinophilic granulomatosis with polyangiitis, and eosinophilic chronic rhinosinusitis. (nih.gov)
  • Churg A. Pulmonary angiitis and granulomatosis revisited. (medscape.com)
  • For patients with GPA that is refractory to remission induction therapy, add IVIG to current therapy. (medscape.com)
  • The randomized, double-blind, placebo-controlled study was designed to assess Imbruvica in combination with Rituxan versus placebo plus Rituxan in 150 patients with either relapsed/refractory (r/r) disease or previously untreated WM. (genengnews.com)
  • 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis. (medscape.com)
  • [ 1 ] Granulomatosis with polyangiitis It is thought to be an autoimmune inflammatory process affecting endothelial cells. (patient.info)
  • The signs found in granulomatosis with polyangiitis occur as a result of the inflammation of the small vessels and may affect any part of the body. (patient.info)
  • Available at https://nieuws.kuleuven.be/en/content/2014/mysterious-esophagus-disease-is-autoimmune-after-all . (medscape.com)
  • Granulomatosis with polyangiitis is capable of mimicking numerous other diseases and it is the presence of two or more of the above signs and/or symptoms which must signal the possibility of the diagnosis. (patient.info)
  • Relapsed or refractory, low grade or follicular, CD20-positive B-cell NHL as a single agent. (nih.gov)
  • La información más reciente sobre el nuevo Coronavirus de 2019, incluidas las clínicas de vacunación para niños de 6 meses en adelante. (stanfordchildrens.org)