Vasculitides are uncommon disorders, characterized by inflammation of the blood vessels resulting either in ischemia or hemorrhage. They are commonly classified as small-, medium-, or large-vessel vasculitides. Antineutrophil cytoplasmic antibody-associated vasculitis is an important group of small-vessel vasculitis. This group includes granulomatous vasculitides, namely, granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and nongranulomatous vasculitis, namely, microscopic polyangiitis (MPA). Classic polyarteritis nodosa (PAN) is a granulomatous medium-vessel vasculitis. This review discusses the classification, etiopathogenesis, clinical features, and management of GPA, MPA, EGPA and PAN.
Key points
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Systemic vasculitides are a group of disorders characterized by inflammation of the blood vessels.
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Granulomatosis with polyangiitis (GPA) is characterized by granulomatous inflammation of upper and lower airways, and by vasculitis of small and medium vessels, of which glomerulonephritis is common.
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Glomerulonephritis and lung hemorrhage are common manifestations of microscopic polyangiitis (MPA).
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The common presentations of polyarteritis nodosa (PAN) are in form of constitutional symptoms, with gastrointestinal (GI), nervous system, and cardiac involvement.
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Treatment is in form of immunosuppression and depends on the type of clinical presentation.
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