RSS Feeds
Resistant Behçet Disease Responsive to Anakinra
- Costantino Botsios, MD, PhD;
- Paolo Sfriso, MD, PhD;
- Antonio Furlan, MD;
- Leonardo Punzi, MD, PhD; and
- Charles A. Dinarello, MD
- From the University of Padova, Padova 35122, Italy, and University of Colorado Health Sciences Center, Denver, CO 80262.
Background: Behçet disease is a systemic disorder with recurrent fever, leukocytosis, myalgias, aphthous and genital ulcers, uveitis, and skin lesions. The presence of vasculitis worsens the prognosis, indicating life-threatening thrombophlebitis, arterial aneurysms, and vascular occlusion (1). Because of its episodic inflammatory nature, Behçet disease is probably an autoinflammatory disease (2).
Objective: To describe a woman with severe Behçet disease who was resistant to conventional therapy as well as infliximab therapy but was successfully treated with interleukin 1 (IL-1) blockade with anakinra.
Case Report: A 75-year-old woman was referred to our department for further management of treatment-resistant Behçet disease. Behçet disease was diagnosed at age 63 years, according to the criteria of the International Study Group for Behçet's Disease. The patient had recurrent fever, oral and genital ulcers, a positive pathergy test result, anemia, and elevated erythrocyte sedimentation rate and C-reactive protein level, but was negative for the HLA-B51 allele. Treatment with high-dose prednisone (50 mg/d) and cyclosporine (5 mg/kg of body weight) resulted in a partial reduction in disease severity. Cyclosporine …
