Chondritis of the ear, MI from vasculitis, and aseptic meningitis and nonhealing granulomas of the CNS may occur. Eventually, a disseminated vascular phase may develop with necrotizing inflammatory skin lesions, pulmonary infiltrates and later lesions with cavitation, diffuse leukocytoclastic vasculitis, and focal glomerulitis that may progress to generalized crescentic glomerulonephritis with hypertension and uremia. Occasionally, the disease is limited to pulmonary involvement. Renal involvement is the hallmark of generalized disease. Urinalysis shows proteinuria, hematuria, and RBC casts.
Renal impairment is inevitable without immediate appropriate therapy.
Serum complement levels are normal or elevated. The ESR is elevated. Leukocytosis is present. Anemia may be profound. Antinuclear antibodies and LE cells are absent.