Myositis and scleritis associated with Behçet's disease: an atypical presentation

Abstract

We report a case of an 18-year-old female, who was diagnosed as having systemic Behçet's disease with chief complaints of vision loss in the right eye and redness and pain in both eyes. In addition to an anterior necrotizing scleritis, she had several attacks of retrobulbar neuritis and lateral rectus myositis. She responded well to i.v. and topical prednisolone and her subsequent attacks were treated with i.v. cyclophosphamide (500 mg on presentation and on Days 7, 10, 12, 15, and 20; the cycle was repeated every 20 days). She benefited on this treatment and her vision improved dramatically. Her final acuity of 20/50 was thought to be due to presumed previous attacks of optic neuritis and anterior segment inflammation. During the five-month follow-up period, she had no other attacks. Ischemia and inflammation are common in Behçet's disease and sometimes thrombosis and vasculitic changes may cause a decrease in vision. In this case, the immunosuppressive therapy helped attenuate the inflammatory vasculitic episodes during the disease course.