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Respiratory tract involvement in granulomatosis with polyangiitis and microscopic polyangiitis

Talmadge E King, Jr, MD
Section Editors
Kevin R Flaherty, MD, MS
Richard J Glassock, MD, MACP
Deputy Editor
Helen Hollingsworth, MD


"Classic" granulomatosis with polyangiitis (GPA, Wegener's) is a form of systemic vasculitis (polyangiitis) with necrotizing granulomatous inflammation of the upper and lower respiratory tracts, systemic necrotizing vasculitis, and necrotizing glomerulonephritis [1,2].

Microscopic polyangiitis (MPA) is a necrotizing vasculitis without granulomatous inflammation that predominantly affects small vessels (ie, capillaries, venules, or arterioles) and can present with pulmonary capillaritis or in the context of interstitial lung disease [3]. Both MPA and GPA are considered ANCA-associated vasculitides, although ANCA testing may be negative in some patients.

In the past, the term "limited" GPA was used to describe patients with clinical findings isolated to the upper respiratory tract or lungs, which occurs in approximately one-fourth of cases. The term was confusing, as "limited" disease could be severe. The current practice is to describe the locations of organ involvement and the severity of disease at each location. Thus, a patient can have "severe" disease in the lungs in the presence of antineutrophil cytoplasmic antibody (ANCA)-associated diffuse alveolar hemorrhage with mild extrapulmonary involvement [4]. Many patients with disease limited to upper respiratory tract or lungs subsequently develop systemic involvement [5].

The Boards of Directors of the American College of Rheumatology (ACR), American Society of Nephrology (ASN), and the European League Against Rheumatism (EULAR) have recommended that the name Wegener's granulomatosis be changed to granulomatosis with polyangiitis (Wegener's), abbreviated as GPA. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis", section on 'New terminology'.)

Other organ systems that may be involved in GPA include [1]:

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Literature review current through: Nov 2017. | This topic last updated: Oct 12, 2017.
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