Nationwide epidemiological survey of 169 patients with adult Still's disease in Japan

• Published in: Modern rheumatology Vol. 25; no. 3; pp. 393 - 400
• Main Authors: Asanuma, Yu Funakubo, Mimura, Toshihide, Tsuboi, Hiroto, Noma, Hisashi, Miyoshi, Fumihiko, Yamamoto, Kazuhiko, Sumida, Takayuki
• Format: Journal Article
• Language: English
• Published: United States Informa Healthcare 01.05.2015; Taylor & Francis
• Subjects: Adult; Adult Still's disease; Age of Onset; Aged; Antirheumatic Agents - therapeutic use; Arthritis; Drug therapy; Epidemiology; Exanthema - drug therapy; Female; Ferritin; Fever - drug therapy; Glucocorticoids - therapeutic use; Humans; Japan - epidemiology; Male; Medical prognosis; Medical treatment; Methotrexate; Methotrexate - therapeutic use; Middle Aged; Multicenter study; Prevalence; Prognosis; Questionnaires; Remission Induction; Rheumatology; Still's Disease, Adult-Onset - diagnosis; Still's Disease, Adult-Onset - drug therapy; Still's Disease, Adult-Onset - epidemiology; Tocilizumab; Japan; Methotrexate; Tocilizumab; Multicenter study; Adult Still's disease; Ferritin
• ISSN: 1439-7595; 1439-7609
• DOI: 10.3109/14397595.2014.974881

Abstract Objectives. A nationwide survey was conducted to assess the number of patients, clinical aspects, treatment, and prognosis of adult Still's disease (ASD) in Japan. Methods. A primary questionnaire was sent to randomly selected medical institutions in order to estimate the number of patients. We sent a secondary questionnaire to the same institutions to characterize the clinical manifestations and treatment of ASD. Results. The estimated prevalence of ASD was 3.9 per 100,000. Analysis of 169 patients showed a mean age at onset of 46 years. The main clinical symptoms were fever, arthritis, and typical rash in agreement with previous surveys. Oral glucocorticoids were used to treat 96% of the patients, while methotrexate was used in 41% and biological agents were used in 16%. Lymphadenopathy and macrophage activation syndrome were significantly associated with increased risk of relapse (P < 0.05, each). Patients who achieved remission after tocilizumab therapy had significantly longer disease duration (6.2 years) than patients who did not (1.9 years) (p < 0.05). Conclusions. The 2010-2011 nationwide survey of ASD identified important changes in treatment and improvement of prognosis compared with previous surveys.