Case history
Case history
A previously healthy 61-year-old woman presents with a 3-month history of sinusitis and nasal drainage. She has noted only marginal, temporary improvement despite multiple courses of antibiotics. The nasal drainage is purulent and frequently hemorrhagic. She also has a 2-week history of migratory joint pain, mainly affecting wrists, knees, and ankles. She does not describe joint swelling. She reports having less energy and has lost 10 pounds in weight over the past 2 months. She has no respiratory, urinary, neurologic or other symptoms. Bleeding and inflammation of the nasal mucosa is noted, along with tenderness to percussion over both maxillary sinuses. The remainder of the physical examination is unremarkable. In-office urinalysis reveals 3+ microscopic hematuria and 2+ proteinuria.
Other presentations
These may include upper respiratory tract manifestations (e.g., otitis, deafness, nasal septal perforation, saddle nose deformity, subglottic stenosis, oral and nasal ulcers), lower respiratory tract manifestations (e.g., pulmonary infiltrates, pulmonary hemorrhage, endobronchial lesions), constitutional features (e.g., fever, night sweats, malaise, anorexia), neurologic manifestations (e.g., mononeuritis multiplex, peripheral sensorimotor neuropathy, cranial neuropathy, pachymeningitis, cerebral mass lesions), ocular manifestations (e.g., conjunctivitis, episcleritis, scleritis, proptosis, diplopia, nasolacrimal duct inflammation, retinal vasculitis, uveitis), skin manifestations (e.g., palpable purpura, nodules, hemorrhagic and ulcerative skin lesions), and musculoskeletal features (e.g., myalgias, myositis, arthritis).[1][2][3]
Less commonly, gastrointestinal, cardiac, breast, lower genitourinary tract, or other organ involvement may occur. The vast majority of patients will have involvement of at least two organ systems at the time of diagnosis. There is also an increased incidence of venous thromboembolism, particularly in association with periods of active disease.[4][5]
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