Case ReportsInterleukin 6 Blockade for Hyperimmunoglobulin D and Periodic Fever SyndromeShendi, Hiba M. MD, MBBS, MRCPCH, MSc, FRCpath; Devlin, Lisa A. MD, MBBCh BAO, MRCPCH, FRCPath; Edgar, John David BSc, FRCP, FRCPathAuthor Information From the Royal Victoria Hospital, Belfast, United Kingdom The authors declare no conflict of interest. Correspondence: Hiba M. Shendi, MD, MBBS, MRCPCH, MSc, FRCPath, Royal Victoria Hospital, Grosvenor Rd,Belfast, BT12 6BA United Kingdom. E-mail: [email protected]. JCR: Journal of Clinical Rheumatology: March 2014 - Volume 20 - Issue 2 - p 103-105 doi: 10.1097/01.RHU.0000442576.41537.de Buy Metrics Abstract Hyperimmunoglobulin D and periodic fever syndrome (HIDS) is a rare, autoinflammatory condition caused by mutations in the mevalonate kinase gene. There is no standard treatment for HIDS, and randomized controlled trials are lacking. Corticosteroids, colchicine, nonsteroidal anti-inflammatory drugs, statins, and cyclosporine are of limited efficacy in controlling this condition. Recent case reports suggest that most patients respond to etanercept or anakinra. Interleukin 6 blockade in HIDS has not been described. We report the case of a 13-year-old girl with HIDS, who failed to respond to colchicine, corticosteroids, etanercept, and anakinra but was successfully treated with the anti–IL-6 monoclonal antibody, tocilizumab. © 2014 by Lippincott Williams & Wilkins, Inc.