title: Efficacy and long-term follow-up of IL-1R inhibitor anakinra in adults with Still's disease: a case-series study.
abstract: BACKGROUND
  To assess the efficacy and safety of the interleukin-1 receptor (IL-1R) inhibitor anakinra in adult patients with refractory Still's disease.
  
  
  METHODS
  Twenty-five patients (13 males and 12 females, median age 32 years, median disease duration seven months) with Still's disease were treated with subcutaneous injections of anakinra (100 mg/day). Treatment was given as adjunct therapy in 16 patients and as standalone in 9 patients for a median time of 15 months (range 1.5-71). The clinical and laboratory parameters during follow-up were recorded.
  
  
  RESULTS
  In 84% of patients the clinical activity resolved completely within a few days (median time 0.2 months), and response was maintained until the last visit in all but one patient. A complete response of all disease-related symptoms (clinical and laboratory) occurred subsequently within a median time of three months in 80% of patients. A partial clinical and laboratory improvement was shown in 12% and 16% of patients, respectively. The Visualized Analogue Scale and Health Assessment Questionnaire scores significantly decreased during treatment. The proportion of patients achieving the American College of Rheumatology 20 (ACR20) score (20% improvement) was 82% at one month and improved to 100% at one year. The mean oral corticosteroid dose was significantly reduced at each visit. Anakinra was discontinued due to unresponsiveness in one patient and due to relapsing disease in another. Treatment was also withdrawn in three patients with severe skin reactions (urticaria). Seven patients experienced an infection during follow-up.
  
  
  CONCLUSIONS
  The rapid and sustained response in the majority of our patients encourages the use of anakinra in adults with Still's disease.
authors.affiliation: Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Mikras Asias Street 75, Goudi 11527, Athens, Greece. katerina_laskari@yahoo.gr
authors.forename: Katerina
authors.initials: K
authors.lastname: Laskari
authors.forename: Athanasios G
authors.initials: AG
authors.lastname: Tzioufas
authors.forename: Haralampos M
authors.initials: HM
authors.lastname: Moutsopoulos
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018501:Antirheumatic Agents / Q000008:administration & dosage* / Q000009:adverse effects; D001171:Arthritis, Juvenile / Q000188:drug therapy*; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007279:Injections, Subcutaneous; D053590:Interleukin 1 Receptor Antagonist Protein / Q000008:administration & dosage* / Q000009:adverse effects; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage / Q000009:adverse effects; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D013256:Steroids / Q000008:administration & dosage / Q000009:adverse effects; D016706:Still's Disease, Adult-Onset / Q000188:drug therapy*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein; D013256:Steroids; D008727:Methotrexate