Last update:
04-Apr-2017

Pauci-immune glomerulonephritis, which is the most common cause of aggressive glomerulonephritis, may occur as a renal-limited disease or as a component of systemic necrotizing small-vessel vasculitis. It is characterized histologically by paucity of staining for immunoglobulins, and by immunofluorescence along with fibrinoid necrosis and crescent formation on light microscopy. The vast majority of patients have antineutrophil cytoplasmic antibodies (ANCA) in their circulation, which participate in the pathogenesis of the disease. Pauci-immune glomerulonephritis often manifests with a rapid deterioration in kidney function. This may be accompanied by distinctive clinical features of systemic necrotizing small-vessel vasculitis of one the following clinical phenotypes: Microscopic polyangiitis, granulomatosis with polyangiitis or eosinophilic granulomatosis with polyangiitis. Small vessel vasculitis associated with ANCA may present with a wide spectrum of vasculitic manifestations in various organ systems, both at the time of clinical presentation and during the course of the disease. ANCA specificity is associated with distinct clinical syndromes each with a different prognostic profile. The key element in the management of patients with ANCA associated vasculitis, with or without glomerulonephritis, is clinical acumen, which results in timely diagnosis. Speed in diagnosis is crucial for the timely institution of immunosuppressive therapy aimed at removing circulating autoantibodies and quelling the inflammatory process.

Key words: ANCA, Autoantibodies, Glomerulonephritis, Management, Vasculitis.