Abstract
In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the...
Highlights
Granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) are termed the antineutrophil cytoplasmic antibody (ANCA)associated vasculitides (AAVs).[1]
In 2009 the European League Against Rheumatism (EULAR) published recommendations for managing primary small and medium vessel vasculitis which included the management of AAV.[10]
The publication of 1691 papers in the past 5 years on primary systemic vasculitis in internal medicine, rheumatology and nephrology journals, as well as the licensing of rituximab for AAV, make this an opportune time to update the recommendations with an AAV focus
Summary
Granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) are termed the antineutrophil cytoplasmic antibody (ANCA)associated vasculitides (AAVs).[1] GPA, MPA and EGPA have respective annual incidence rates of 2.1–14.4, 2.4–10.1 and 0.5–3.7 per million in Europe, and the prevalence of AAV is estimated at to be 46–184 per million.[2,3,4,5,6,7,8] The 5-year survival rates for GPA, MPA and EGPA are estimated to be 74–91%, 45–76% and 60–97%, respectively.[9]
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