Efficacy of Anakinra in Refractory Adult-Onset Still's Disease

Francisco Ortiz-Sanjuán, Ricardo Blanco, Leyre Riancho-Zarrabeitia, Santos Castañeda, Alejandro Olivé, Anne Riveros, María.L. Velloso-Feijoo, Javier Narváez, Inmaculada Jiménez-Moleón, Olga Maiz-Alonso, Carmen Ordóñez, José A. Bernal (+20 others)
2015 Medicine  
Adult-onset Still's disease (AOSD) is often refractory to standard therapy. Anakinra (ANK), an interleukin-1 receptor antagonist, has demonstrated efficacy in single cases and small series of AOSD. We assessed the efficacy of ANK in a series of AOSD patients. Multicenter retrospective open-label study. ANK was used due to lack of efficacy to standard synthetic immunosuppressive drugs and in some cases also to at least 1 biologic agent. Forty-one patients (26 women/15 men) were recruited. They
more » ... d a mean age of 34.4 AE 14 years and a median [interquartile range (IQR)] AOSD duration of 3.5 [2-6] years before ANK onset. At that time the most common clinical features were joint manifestations 87.8%, fever 78%, and cutaneous rash 58.5%. ANK yielded rapid and maintained clinical and laboratory improvement. After 1 year of therapy, the frequency of joint and cutaneous manifestations had decreased to 41.5% and to 7.3% respectively, fever from 78% to 14.6%, anemia from 56.1% to 9.8%, and lymphadenopathy from 26.8% to 4.9%. A dramatic improvement of laboratory parameters was also achieved. The median [IQR] prednisone dose was also reduced from 20 [11.3-47.5] mg/day at ANK onset to 5 [0-10] at 12 months. After a median [IQR] follow-up of 16 [5-50] months, the most important side effects were cutaneous manifestations (n ¼ 8), mild leukopenia (n ¼ 3), myopathy (n ¼ 1), and infections (n ¼ 5). ANK is associated with rapid and maintained clinical and laboratory improvement, even in nonresponders to other biologic agents. However, joint manifestations are more refractory than the systemic manifestations. (Medicine 94(39):e1554) Abbreviations: ACR = American College of Rheumatology, ANK = anakinra, AOSD = adult-onset still disease, CRP = c-reactive protein, DMARD = disease-modifying antirheumatic drugs, ESR = erythrocyte sedimentation rate, FDA = Food and Drug Administration, IFN-g = interferon-g, IL-1 = interleukin 1, IL-6 = interleukin-6, IL-18 = interleukin 18, IQR = interquartile range, NSAIDS = non-steroidal anti-inflammatory drugs, SD = standard deviation, TNF-a = tumor necrosis factor-a. This is an open access article distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0, where it is permissible to download, share and reproduce the work in any medium, provided it is properly cited. The work cannot be changed in any way or used commercially. Ã In 2 patients from this series ANK was started in combination with disease modifying antirheumatic drugs (DMARDs) (both patients were on treatment with conventional DMARDs before the onset of ANK). In another 2 patients, ANK was initially used as monotherapy and conventional DMARD therapy was added in the follow-up because of persistence of symptoms. þ Some patients received more than 1 TNF inhibitor.
doi:10.1097/md.0000000000001554 pmid:26426623 pmcid:PMC4616841 fatcat:u3id35yclnff7kydnsoocnjd6a