The typical patient is male about 50 years old.
General systemic features:
(1) fever
(2) anorexia and weight loss
(3) malaise
Respiratory:
(1) pulmonary hemorrhage
(2) alveolar capillaritis on biopsy
(3) interstitial fibrosis
(4) pleurisy
(5) occasionally asthma
Renal:
(1) rapidly progressive glomerulonephritis with necrotizing glomerulonephritis on biopsy and deterioration in renal function
(2) hypertension
Skin:
(1) purpura
(2) splinter hemorrhages
(3) skin rash, especially over the extremities
(4) rarely small punched-out ulcers
Gastrointestinal Tract:
(1) abdominal pain
(2) GI bleeding
Neurologic:
(1) peripheral neuropathy
Ocular:
(1) conjunctivitis
(2) uveitis
(3) retinal vasculitis
(4) scleritis
Head and neck:
(1) mucositis, with lesions in the ears, nose and/or throat
Cardiovascular:
(1) vasculitis
(2) rarely microaneurysms
Musculoskeletal:
(1) myalgias
(2) arthralgias
(3) arthritis
Laboratory findings:
(1) elevation in ESR and CRP
(2) elevated serum creatinine
(3) usually pANCA positive; occasionally cANCA positive; sometimes ANCA negative
(4) negative for HbsAg
(5) anemia
(6) pulmonary siderophages in the BAL
(7) urine sediment consistent with glomerulonephritis
