Clinical study on death in Behçet's disease.

We were able to identify 7 patients who died of Behçet's disease, among 2,200 patients registered at the Behçet's Disease Specialty Clinic of Severance Hospital from November 1983 to October 1992. Six were male and one female. Age of death was 31 to 55 years with the mean age of 39.1. The age of onset was 24 to 54 years with the mean age of 32.6. The mean duration of illness was 6.5 years. The most frequent initial symptom was oral ulcer, with the most common type being the incomplete type in Shimizu's classification. Positive pathergy tests were found in all the 5 patients in whom the test was performed out of 7 patients. Various modalities of treatments such as oral steroid, colchicine, azathioprine, colectomy, and pacemaker insertion were attempted without outstanding therapeutic effects. The causes of death were gastrointestinal bleeding, bowel perforation, superior and inferior vena cava syndrome, aortic regurgitation, cerebrovascular disease, sepsis, and lung abscess.