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, María V. Hernández, Walter A. Sifuentes-Giraldo, Catalina Gómez-Arango, Eva Galíndez-Agirregoikoa, Juan Blanco-Madrigal, Vera Ortiz-Santamaria, Jordi del Blanco-Barnusell, Juan R. De Dios, Mireia Moreno, Jordi Fiter, Marina de los Riscos, Patricia Carreira, María J. Rodriguez-Valls, M. Carmen González-Vela, Vanesa Calvo-Río, Javier Loricera, Natalia Palmou-Fontana, Trinitario Pina, Javier Llorca, Miguel A. González-Gay

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 had a mean age of 34.4 ± 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 = 8), mild leukopenia (n = 3), myopathy (n = 1), and infections (n = 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.