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

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.

Keywords:

• Adult Still's disease
• Ferritin
• Methotrexate
• Multicenter study
• Tocilizumab

Acknowledgments

We thank Hiromitsu Asashima, Chinatsu Takai, Shinya Hagiwara, Chihiro Hagiya, Masahiro Yokosawa, and Tomoya Hirota from the Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, for data acquisition.

Funding

This study was supported by research grants from the Ministry of Education, Culture, Sports, Science and Technology of Japan, and Health and Labour Sciences Research Grants for research on intractable diseases (The Research Team for Autoimmune Diseases) from the Ministry of Health, Labour and Welfare of Japan.

Conflict of interest

None.