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Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a multisystem disease of unknown etiology characterized by vasculitis and necrotizing granulomatous inflammation of the small arteries and veins. Classic generalized GPA is a triad of necrotizing granulomas of the upper and lower respiratory tract, systemic necrotizing small vessel granulomatous vasculitis, and glomerulonephritis (usually focal segmental). GPA most often occurs in patients in middle age and of Northern European descent (80%-90% of cases), but cases have been reported in children and older adults.
The disease typically presents with upper airway disease and nonspecific complaints such as malaise, arthralgias, fever, and weight loss. Respiratory tract symptoms include sinusitis, otitis media, rhinorrhea, cough, sputum production, chest pain, dyspnea, and hemoptysis.
The disease presents variably on a continuum from limited involvement (which may remain limited for variable periods of time) to a more generalized form with involvement of the upper and lower respiratory tract, kidney, skin, and other organs. Untreated, the generalized form is fatal in less than 2 years; however, long-term remissions are now possible in more than 90% of patients with the advent of cytotoxic therapy.
Complications of GPA include pericarditis, coronary arteritis, myocardial infarction, pancarditis, granulomatous valvulitis, mononeuritis multiplex, and polyneuritis. Laryngotracheal stenosis with stridor can be a complication of GPA and initially may be misdiagnosed as asthma.
usmle
granulomatosis with polyangiitis
wegener's granulomatosis
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