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ANCA Vasculitis

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6 papers 0 to 25 followers
By Isabel Acosta-Ochoa Nephrology senior staff. Valladolid. Spain
Divi Cornec, Emilie Cornec-Le Gall, Ulrich Specks
The prognosis of the antineutrophil cytoplasmic antibody associated vasculitides (AAV), microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), has been fundamentally improved over the last five decades by the use of glucocorticoids and immunosuppressants, turning them from consistently fatal diseases into chronic conditions. The long-term course is now largely determined by the frequency of disease flares and by accruing damage caused by disease activity and treatment-related comorbidities...
January 12, 2017: Nephrology, Dialysis, Transplantation
Andrea Aguilar
No abstract text is available yet for this article.
July 18, 2016: Nature Reviews. Nephrology
Anoek A E de Joode, Jan Stephan F Sanders, Abraham Rutgers, Coen A Stegeman
Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are severe chronic auto-immune diseases in which the small vessels are inflamed. Nowadays, in the majority of patients disease can be brought into remission with cyclophosphamide and corticosteroids. However, depending upon disease characteristics patients with AAV have a risk of 29-60% to experience relapses of disease within 5 years despite maintenance therapy after induction of remission with less toxic agents, such as azathioprine, methotrexate or mycophenolate mofetil...
April 2015: Nephrology, Dialysis, Transplantation
Kerstin Westman, Oliver Flossmann, Gina Gregorini
Patients with generalized ANCA-associated small vessel vasculitis (AAV) have a very poor outcome if the ANCA-associated vasculitis is not diagnosed, evaluated and treated properly. The introduction of treatment with immunosuppressive therapy has improved patient survival dramatically but with considerable side effects. Besides, almost 50% of surviving patients experience a relapse of vasculitis. Since 1995, the European Vasculitis Society (EUVAS) has designed and conducted several clinical trials on patients with AAV independently of pharmaceutical companies...
April 2015: Nephrology, Dialysis, Transplantation
Ulf Schönermarck, Elena Csernok, Wolfgang L Gross
Anti-neutrophil cytoplasmic autoantibodies (ANCA) with specificity for proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA) are a defining feature of ANCA-associated vasculitides (AAV). They play a pivotal role in disease pathophysiology and have strongly improved early diagnosis and treatment of these infrequent, but potentially fatal diseases. Neutrophils and their products are major players in initiating the autoimmune response and tissue destruction in vasculitic as well as granulomatous inflammation. This review highlights recent findings on old and novel players (ANCA, neutrophils, neutrophil extracellular traps, fibroblasts, immune cells and complement) and puts them into context with the current understanding of disease mechanisms in AAV...
April 2015: Nephrology, Dialysis, Transplantation
J S F Sanders, W H Abdulahad, C A Stegeman, C G M Kallenberg
Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are autoimmune diseases in which the small vessels are inflamed. Clinical observations suggest a pathogenic role for ANCA. Such a role is supported by in vitro experimental data and animal models, particularly for myeloperoxidase-ANCA. An in vivo pathogenic role of ANCA directed to proteinase 3 has, however, not been fully substantiated. Additionally, the pathogenic role of B cells, T cells, and the alternative pathway of complement in AAV have been elucidated...
2014: Nephron. Clinical Practice
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