Ortiz-Sanjuán, FranciscoBlanco, RicardoRiancho-Zarrabeitia, LeyreCastañeda, SantosOlivé, AlejandroRiveros, AnneVelloso-Feijoo, María LNarváez, JavierJiménez-Moleón, InmaculadaMaiz-Alonso, OlgaOrdóñez, CarmenBernal, José AHernández, María VSifuentes-Giraldo, Walter AGómez-Arango, CatalinaGalíndez-Agirregoikoa, EvaBlanco-Madrigal, JuanOrtiz-Santamaria, Veradel Blanco-Barnusell, JordiDe Dios, Juan RMoreno, MireiaFiter, Jordide los Riscos, MarinaCarreira, PatriciaRodriguez-Valls, María JGonzález-Vela, M CarmenCalvo-Río, VanesaLoricera, JavierPalmou-Fontana, NataliaPina, TrinitarioLlorca, JavierGonzález-Gay, Miguel A2017-05-032017-05-032015-09Ortiz-Sanjuán F, Blanco R, Riancho-Zarrabeitia L, Castañeda S, Olivé A, Riveros A, et al. Efficacy of Anakinra in Refractory Adult-Onset Still's Disease: Multicenter Study of 41 Patients and Literature Review. Medicine (Baltimore). 2015 ; 94(39):e15540025-7974http://hdl.handle.net/10668/2626Adult-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.enAdultoQuimioterapia combinadaFemeninoHumanosInmunosupresoresProteína antagonista del receptor de interleucina 1MasculinoMediana edadPrednisonaEstudios retrospectivosEnfermedad de Still del adultoMedical Subject Headings::Persons::Persons::Age Groups::AdultMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Therapeutics::Drug Therapy::Drug Therapy, CombinationMedical Subject Headings::Check Tags::FemaleMedical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::HumansMedical Subject Headings::Chemicals and Drugs::Chemical Actions and Uses::Pharmacologic Actions::Physiological Effects of Drugs::Immunologic Factors::Immunosuppressive AgentsMedical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Peptides::Intercellular Signaling Peptides and Proteins::Cytokines::Interleukin 1 Receptor Antagonist ProteinMedical Subject Headings::Check Tags::MaleMedical Subject Headings::Persons::Persons::Age Groups::Adult::Middle AgedMedical Subject Headings::Chemicals and Drugs::Polycyclic Compounds::Steroids::Pregnanes::Pregnadienes::Pregnadienediols::PrednisoneMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Case-Control Studies::Retrospective StudiesMedical Subject Headings::Diseases::Immune System Diseases::Autoimmune Diseases::Arthritis, Rheumatoid::Still's Disease, Adult-Onsetresearch article26426623open access10.1097/MD.00000000000015541536-5964PMC4616841