Pulmonary vasculitides include a broad variety of disorders having in common inflammation and destruction of the blood vessels within the lung. Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is an antineutrophil cytoplasmic antibody (ANCA)-Associated small-vessel vasculitis that affects a number of organ systems, and is the most common of the ANCAassociated vasculitides. GPA affects a broad age range, most commonly presenting in middle-Aged adults. The classic triad of GPA, the combination of upper-Airway disease (sinusitis, otitis media, ulcerations, tracheobronchial stenoses), lower-respiratory tract disease, and glomerulonephritis, is not always evident at presentation. Imaging manifestations of GPA commonly include multiple, bilateral nodules or masses that often show cavitation or necrosis. Less commonly, diffuse lung opacity resulting from alveolar hemorrhage, or tracheobronchial stenoses, may be seen. The diagnosis of systemic GPA can often be suggested when c-ANCA/anti-PR-3 antibodies are detected in the proper clinical context, but not infrequently, the diagnosis rests on recognition of a combination of characteristic clinical, laboratory, and imaging findings in combination with histopathologic material from a biopsy. The mainstay of treatment of GPA is corticosteroid therapy in combination with other immunosuppressive agents.
- Granulomatosis with polyangiitis
- Renal failure
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
- Critical Care and Intensive Care Medicine