Efficacy Of Anakinra In Refractory Adult-Onset Still'S Disease: Multicenter Open-Label Study Of 34 Patients

Background Adult-onset Still9s 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