E-Mail karger@karger.com Case Report Dermatology 2014;228:211–214 DOI: 10.1159/000358125 Inhibition of Interleukin-1 by Canakinumab as a Successful Mono-Drug Strategy for the Treatment of Refractory Behçet’s Disease: A Case Series Antonio Vitale a Donato Rigante b Francesco Caso a, c Maria Giuseppina Brizi a Mauro Galeazzi a Luisa Costa d Rossella Franceschini e Orso Maria Lucherini a Luca Cantarini a a Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, b Institute of Pediatrics, Università Cattolica Sacro Cuore, Rome, c Rheumatology Unit, Department of Medicine, University of Padua, Padua, d Rheumatology Research Unit, Department of Clinical and Experimental Medicine, University Federico II, Naples, and e Ophthalmology and Neurosurgery Department, University of Siena, Siena, Italy decided according to the individual patient, severity or recurrence of clinical manifesta- tions and major organ involvement. © 2014 S. Karger AG, Basel Introduction Behçet’s disease (BD) is a rare multisys- tem inflammatory disorder clinically char- acterized by recurrent ulcers of the mouth and genitals, chronic uveitis and heteroge- neous skin lesions, such as pseudofolliculi- tis, papulopustular eruption, erythema no- dosum-like and erythema multiforme-like lesions. Other various manifestations in- clude positive pathergy test, arthritides, ar- terial and/or venous thrombosis, central or peripheral nervous system disease and gas- trointestinal vasculitis [1]. Generally BD is not a life-threatening condition, although mortality can be associated with vascular- thrombotic and neurological manifesta- tions [2]. Despite several available thera- peutic options, the treatment is often chal- lenging and currently tailored according to the severity of each clinical feature. An- ti-tumor necrosis factor α (anti-TNF-α) Key Words Behçet’s disease · Uveitis · Interleukin-1 · Canakinumab Abstract Recommendations related to ocular, muco- sal and cutaneous involvement of Behçet’s disease (BD) are mainly evidence-based, but in cases of vascular, neurological and gastro- intestinal involvement there are no guide- lines to define the best treatment strategy. We report three adult patients with BD, who received an interleukin-1β inhibitor by subcutaneous injections, canakinumab (at the dosage of 150 mg every 6 weeks), after failure shown by corticosteroids and differ- ent combinations of immunosuppressant agents. The prompt and sustained clinical ef- ficacy demonstrated by canakinumab as a monotherapy supports the opportunity of using this specific anti-interleukin-1β agent as a valid therapeutic option for resistant or refractory BD. Open trials and observation- al studies should be performed to test canakinumab efficacy on a larger number of patients. The most appropriate dosage and intervals between administrations should be Received: November 2, 2013 Accepted after revision: December 18, 2013 Published online: March 12, 2014 Luca Cantarini, MD, PhD Rheumatology Unit, Policlinico Le Scotte University of Siena Viale Bracci 1, IT–53100 Siena (Italy) E-Mail cantariniluca  @  hotmail.com © 2014 S. Karger AG, Basel 1018–8665/14/2283–0211$39.50/0 www.karger.com/drm agents have been employed in compelling cases as an add-on therapy combined with corticosteroids and/or immunomodulat- ing drugs, such as methotrexate, azathio- prine, chlorambucil and cyclophospha- mide [3–7]. In addition, resistance and loss of efficacy have been also described [8]. Recently, single case reports and case series provided preliminary encouraging data on successful interleukin-1 (IL-1) in- hibition in patients with BD, leading to in- creasing interest in anti-IL-1 agents [9–12]. In this regard, the IL-1 receptor antagonist anakinra and the human immunoglobulin G1 (IgG1) anti-IL-1β monoclonal antibody canakinumab have been recently proposed as possible therapeutic tools in BD. Herein we report three BD patients suc- cessfully treated with canakinumab admin- istered as monotherapy, confirming that inhibition of the proinflammatory actions of IL-1 is paramount in controlling the clinical sceneries of BD. A. Vitale and D. Rigante contributed equally to this paper. Downloaded by: 198.143.37.65 - 4/9/2016 2:37:16 AM