TY - CHAP M1 - Book, Section TI - Chapter 32. Granulomatosis with Polyangiitis (Wegener Granulomatosis) A1 - Stone, John H. A2 - Imboden, John B. A2 - Hellmann, David B. A2 - Stone, John H. PY - 2013 T2 - CURRENT Diagnosis & Treatment: Rheumatology, 3e AB - Three pathologic hallmarks: granulomatous inflammation, vasculitis, and necrosis.Classic clinical features are found in multiple organ systems:Nonspecific constitutional symptoms, such as fatigue, myalgias, weight loss, and fevers.Persistent upper respiratory tract and ear “infections” that do not respond to antibiotic therapy.Orbital pseudotumor, nearly always associated with chronic nasosinus conditions.Migratory pauciarticular or polyarticular arthritis.Nodular or cavitary lung lesions that are misdiagnosed initially as malignancies or infections.Rapidly progressive glomerulonephritis.Antineutrophil cytoplasmic antibody (ANCA) assays are helpful in diagnosis if positive by both immunofluorescence and enzyme immunoassay. A significant minority of patients with granulomatosis with polyangiitis are ANCA-negative, particularly those with “limited” disease. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/04/16 UR - accessmedicine.mhmedical.com/content.aspx?aid=57273174 ER -