Refractory Behçet's Disease with Multi-organ Involvement - Learning from Failure

• Published in: Current rheumatology reviews Vol. 17; no. 3; p. 360
• Main Authors: Santos, Christiana Sieiro, Morales, Clara Moriano, Álvarez, Elvira Díez, Sandoval, Trinidad Pérez
• Format: Journal Article
• Language: English
• Published: United Arab Emirates 01.01.2021
• Subjects: Behcet Syndrome - complications; Behcet Syndrome - drug therapy; Humans; Multiple Organ Failure - complications; Behçet; intestinal; refractory; case report; vasculitis; ulcers
• ISSN: 1875-6360
• DOI: 10.2174/1573403X16999201228205028

Behçet's disease (BD) is a rare systemic vasculitis of unknown etiology with relapsing and remitting course, characterized by triple-symptom complex of recurrent attacks of oral aphthous ulcers, genital ulcers and ocular lesions and other clinical features that include dermatological, cardiovascular, gastrointestinal, and neurological manifestations. The main goal of management is to prevent relapses and suppress inflammation rapidly for major organ involvement that may cause damage and even be fatal. We hereby describe a case of a patient with Behçet's disease followed in our rheumatology consultation over the course of 15 years with multi-organ involvement resistant to several treatments and showed partial results with IL-1 inhibitors, particularly anakinra and canakinumab, while also discussing the current treatments of refractory BD and how early implementation of treatments could make a difference.