TY - CHAP M1 - Book, Section TI - Granulomatosis with Polyangiitis A1 - Stone, John H. A2 - Stone, John H. PY - 2021 T2 - Current Diagnosis & Treatment: Rheumatology, 4e 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.Migratory pauciarticular or polyarticular arthritis.Persistent upper respiratory tract and ear “infections” that do not respond to antibiotic therapy.Orbital pseudotumor, nearly always associated with chronic nasosinus conditions.Nodular or cavitary lung lesions that are misdiagnosed initially as malignancies or infections.Rapidly progressive glomerulonephritis.Antineutrophil cytoplasmic antibody (ANCA) assays are extremely helpful in diagnosis but have significant shortcomings as indicators of disease activity or guides to when to treat. A significant minority of patients with granulomatosis with polyangiitis are ANCA negative, particularly those with “limited” disease. SN - PB - McGraw Hill CY - New York, NY Y2 - 2024/04/16 UR - accessmedicine.mhmedical.com/content.aspx?aid=1180197326 ER -