musculoskeletal and connective tissue disorders
Granulomatosis with Polyangiitis (GPA)
Key Points
In granulomatosis with polyangiitis, vasculitis affects small- and medium-sized vessels in any organ, typically the kidneys (with glomerulonephritis), and upper and lower respiratory tracts with significant necrotizing parenchymal granulomatous inflammation, which is often more striking than the vasculitis. Manifestations can affect various organ systems and often include upper and lower respiratory tract symptoms (eg, recurrent nasal discharge or epistaxis, cough), followed by hypertension and edema (due to kidney involvement). Confirm the diagnosis with antineutrophil cytoplasmic antibodies testing and biopsy. Relapses are common, and treatments can contribute to morbidity. Induce remission with corticosteroids plus an immunosuppressant. Maintain remission withmethotrexate,azathioprine, orrituximaband taper the corticosteroid dose. 