THU0390 Efficacy of Anakinra in Refractory Adult-Onset Still's Disease: Multicenter Open-Label Study of 34 Patients. (10th June 2014)

• Record Type: Journal Article
• Authors: Ortiz Sanjuán, F.M.; Blanco, R.; Olivé, A.; Riveros, A.; Castañeda, S.; Velloso-Feijoo, M.L.; Narváez, F.J.; Jiménez-Moleόn, I.; Ordόñez, C.; Bernal, J.A.; Sifuentes-Giraldo, W.A.; Gόmez-Arango, C.; Galíndez-Agirregoikoa, E.; Ortiz-Santamaria, V.; del Blanco-Barnusell, J.; De Dios, J.R.; Moreno, M.; Fiter, J.; Loricera, J.; Calvo-Río, V.; Pina, T.; González-Gay, M.A.
• Is Part Of: Annals of the rheumatic diseases. Volume 73:Supplement 2(2014)
• Page Start: 316
• Page End: 317
• Publication Date: 2014-06-10
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2014-eular.5607
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: Adult-onset Still's disease (AOSD) is frequently refractory to standard therapy. Anakinra (ANK) is an interleukin-1 (IL-1) receptor antagonist. The ANK has demonstrated efficacy in single cases or in small series of AOSD. We assessed the efficacy of ANK in a multicenter study. Objectives: We assessed the efficacy of ANK in a multicenter study. Methods: Retrospective open-label study of 34 patients with AOSD. ANK was used because of inadequate response to corticosteroids and at least 1 standard synthetic immunosuppressive drug, and in many cases also to biologic agents. Results: Patients (22 women/12 men) had a mean age of 33.1±13.2 years and a median [interquartile range- IQR] AOSD duration of 1.6 [0.1-24] years before ANK onset. At ANK onset, the most frequent clinical manifestations were: joint manifestations (n=30), fever (n=25) and cutaneous manifestations (n=20). In reference to laboratory parameters, high C reactive protein (CRP) (n=31), high erythrocyte sedimentation rate (ESR) (n=27), leukocytosis (n=22) or anemia (n=18). ANK yielded rapid and maintained clinical and laboratory improvement (Table). After one year of ANK therapy, joint manifestations had decreased from 88.2% to 17.6%, fever from 73.5% to 5.9%, cutaneous manifestations from 58.8 to 2.9%, and lymphadenopathy from 20.6% to 0%. Also, a dramatic reduction of laboratory markers of inflammation including CRP, ESR and ferritin was achieved. The median [IQR] dose of prednisone was also reduced from 20 [0-100] mg/day at ANK onset to 5 [0-40] at 12 months. After a median [IQR] follow-up of 16 [1-206] months, the most important side effects were: cutaneous (n=6), mild leukopenia (n=2), myopathy with elevation of muscle enzymes (n=1), phalanx osteomyelitis (n=1) and urinary tract infection (n=1). Conclusions: ANK is associated with rapid and maintained clinical and laboratory improvement in refractory AOSD. However, joint manifestations seem to be more refractory than systemic manifestations. Acknowledgements: This study was supported by a grant from "Fondo de Investigaciones Sanitarias" PI12/00193 (Spain). This work was also partially supported by RETICS Programs, RD08/0075 (RIER) and RD12/0009/0013 from "Instituto de Salud Carlos III" (ISCIII) (Spain). Disclosure of Interest: : None declared DOI: 10.1136/annrheumdis-2014-eular.5607