The Expanding Spectrum of Behçet Syndrome

Abstract

BEHCET syndrome was originally characterized by a diagnostic triad of aphthous stomatitis, genital ulceration, and uveitis. However, the spectrum of this entity has progressively broadened with the recognition of multiple organ system involvement; most frequently, dermal vasculitis and arthritis occur. Other manifestations that occur less commonly include CNS involvement, colitis, phlebitis, and myocarditis.1We report a case of Behcet syndrome with aphthous stomatitis, genital ulceration, polyarthritis, myocarditis, and severe renal involvement with focal necrotizing glomerulonephritis. To our knowledge, it represents the first case of acute glomerulonephritis as a manifestation of Behcet syndrome Report of a Case A 58-year-old man was admitted to Thomas Jefferson University Hospital because of pain in multiple proximal interphalangeal joints and shortness of breath. Since 1967, he had had recurrent aphthous mouth ulcers, intermittent painful swelling of both ankles and the right great toe, and exertional breathlessness. The patient was dyspneic, and his jugular veins