Resistant Behçet Disease Responsive to Anakinra
Authors: Costantino Botsios, Paolo Sfriso, Antonio Furlan, Leonardo Punzi, Charles A. Dinarello
Journal: Annals of Internal Medicine (2008)

Letters19 August 2008Resistant Behçet Disease Responsive to AnakinraCostantino Botsios, MD, PhD, Paolo Sfriso, MD, PhD, Antonio Furlan, MD, Leonardo Punzi, MD, PhD, and Charles A. Dinarello, MDCostantino Botsios, MD, PhDFrom the University of Padova, Padova 35122, Italy, and University of Colorado Health Sciences Center, Denver, CO 80262., Paolo Sfriso, MD, PhDFrom the University of Padova, Padova 35122, Italy, and University of Colorado Health Sciences Center, Denver, CO 80262., Antonio Furlan, MDFrom the University of Padova, Padova 35122, Italy, and University of Colorado Health Sciences Center, Denver, CO 80262., Leonardo Punzi, MD, PhDFrom the University of Padova, Padova 35122, Italy, and University of Colorado Health Sciences Center, Denver, CO 80262., and Charles A. Dinarello, MDFrom the University of Padova, Padova 35122, Italy, and University of Colorado Health Sciences Center, Denver, CO 80262.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-149-4-200808190-00018 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail 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 ...References1. Yazici H, Fresko I, Yurdakul S. Behçet's syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol. 2007;3:148-55. [PMID: 17334337] CrossrefMedlineGoogle Scholar2. Gül A. Behçet's disease as an autoinflammatory disorder. Curr Drug Targets Inflamm Allergy. 2005;4:81-3. [PMID: 15720240] CrossrefMedlineGoogle Scholar3. Touitou I, Magne X, Molinari N, Navarro A, Quellec AL, Picco P, et al. MEFV mutations in Behçet's disease. Hum Mutat. 2000;16:271-2. [PMID: 10980540] CrossrefMedlineGoogle Scholar4. Goldbach-Mansky R, Dailey NJ, Canna SW, Gelabert A, Jones J, Rubin BI, et al. Neonatal-onset multisystem inflammatory disease responsive to interleukin-1beta inhibition. N Engl J Med. 2006;355:581-92. [PMID: 16899778] CrossrefMedlineGoogle Scholar5. Dinarello CA. The many worlds of reducing interleukin-1 [Editorial]. Arthritis Rheum. 2005;52:1960-7. [PMID: 15986340] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAuthors: Costantino Botsios, MD, PhD; Paolo Sfriso, MD, PhD; Antonio Furlan, MD; Leonardo Punzi, MD, PhD; Charles A. Dinarello, MDAffiliations: From the University of Padova, Padova 35122, Italy, and University of Colorado Health Sciences Center, Denver, CO 80262.Disclosures: None disclosed. 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