Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis
- Ronald J Falk, MD
Ronald J Falk, MD
- Allan Brewster Distinguished Professor of Medicine
- Chair, Department of Medicine
- Director, UNC Kidney Center
- Director, Center for Transplant Care
- University of North Carolina-Chapel Hill
- Peter A Merkel, MD, MPH
Peter A Merkel, MD, MPH
- Section Editor — Vasculitis
- Chief, Division of Rheumatology
- University of Pennsylvania
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
- Section Editors
- Richard J Glassock, MD, MACP
Richard J Glassock, MD, MACP
- Editor-in-Chief — Nephrology
- Section Editor — Glomerular Diseases
- Emeritus Professor
- The David Geffen School of Medicine at UCLA
- Gerald B Appel, MD
Gerald B Appel, MD
- Section Editor — Glomerular Diseases
- Professor of Medicine
- Columbia University College of Physicians and Surgeons
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 antibodies" and "Pathogenesis of granulomatosis with polyangiitis and related vasculitides" and "Overview of and approach to the vasculitides in adults".)
CLASSIFICATION AND DIAGNOSTIC CRITERIA
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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- Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. J Am Soc Nephrol 2011; 22:587.
- Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. Ann Rheum Dis 2011; 70:704.
- Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
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- Haas M, Eustace JA. Immune complex deposits in ANCA-associated crescentic glomerulonephritis: a study of 126 cases. Kidney Int 2004; 65:2145.
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- NEW TERMINOLOGY
- CLASSIFICATION AND DIAGNOSTIC CRITERIA
- ACR criteria
- Chapel Hill Consensus Conference criteria
- European Medicines Agency algorithm
- Limitations of classification algorithms
- CLINICAL PRESENTATION
- Common clinical presentations
- Ear, nose, and throat
- Tracheal and pulmonary disease
- Renal manifestations
- - ANCA-negative pauci-immune crescentic glomerulonephritis
- Cutaneous manifestations
- Ophthalmic and orbital manifestations
- Other manifestations
- Nonsevere ANCA-associated vasculitis
- Establishing the diagnosis
- Physical examination
- Laboratory investigation
- Radiographic tests
- Bronchoalveolar lavage
- - Renal histology
- Pauci-immune glomerulonephritis
- Histologic variants
- Histopathologic classification
- - Renal-limited vasculitis
- DIFFERENTIAL DIAGNOSIS
- Concurrent glomerular disease
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS