Nationwide epidemiological survey of 169 patients with adult Still's disease in Japan.
OBJECTIVE
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.
Department of Rheumatology and Applied Immunology, Faculty of Medicine, Saitama Medical University , Saitama , Japan.
Yu Funakubo
YF
Asanuma
Toshihide
T
Mimura
Hiroto
H
Tsuboi
Hisashi
H
Noma
Fumihiko
F
Miyoshi
Kazuhiko
K
Yamamoto
Takayuki
T
Sumida
D000328:Adult; D017668:Age of Onset; D000368:Aged; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D005076:Exanthema / Q000188:drug therapy; D005260:Female; D005334:Fever / Q000188:drug therapy; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use*; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D012074:Remission Induction; D016706:Still's Disease, Adult-Onset / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*
D018501:Antirheumatic Agents; D005938:Glucocorticoids; D008727:Methotrexate
Adult Still's disease; Ferritin; Methotrexate; Multicenter study; Tocilizumab