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Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis

Ronald J Falk, MD
Peter A Merkel, MD, MPH
Talmadge E King, Jr, MD
Section Editors
Richard J Glassock, MD, MACP
Gerald B Appel, MD
Deputy Editors
Albert Q Lam, MD
Monica Ramirez Curtis, MD, MPH


In January 2011, the Boards of Directors of the American College of Rheumatology (ACR), the American Society of Nephrology (ASN), and the European League Against Rheumatism (EULAR) recommended that the name "Wegener's granulomatosis" be changed to "granulomatosis with polyangiitis," abbreviated as GPA [1-3]. This change reflects a plan to gradually shift from honorific eponyms to a disease-descriptive or etiology-based nomenclature.


Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), including renal-limited vasculitis (RLV), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss) [4-6]. All are associated with ANCA and have similar features on renal histology (eg, a focal necrotizing, often crescentic, pauci-immune glomerulonephritis).

The clinical manifestations and diagnosis of GPA, MPA, and RLV are reviewed here. EGPA has a different presentation and prognosis compared with the other forms of AAV. (See "Epidemiology, pathogenesis, and pathology of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)" and "Treatment and prognosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)".)

Issues related to ANCA, the pathogenesis of AAV, and the vasculitides in general are discussed separately. (See "Clinical spectrum of antineutrophil cytoplasmic autoantibodies" and "Pathogenesis of granulomatosis with polyangiitis and related vasculitides" and "Overview of and approach to the vasculitides in adults".)


There have been several attempts to standardize the classification and diagnostic criteria for small-vessel vasculitis [5,7,8]. The International Chapel Hill Consensus Conference (CHCC) criteria, which were revised in 2012, are the mostly commonly cited (table 1). However, as will be described, the classification algorithms have important limitations. (See 'Limitations of classification algorithms' below.)

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Literature review current through: Nov 2017. | This topic last updated: Nov 15, 2016.
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