Multicenter questionnaire survey for sporadic inclusion body myositis in Japan

Orphanet Journal of Rare Diseases

Naoko Matsui¹  Ryuji Kaji¹  Yukie Inamori²  Itsuro Higuchi²  Satoshi Yamashita³  En Kimura³  Hirofumi Kusaka⁴  Madoka Mori-Yoshimura⁵  Toshiaki Takahashi⁶  Satoshi Nakano⁷  Hiroya Ono⁸  Hitoshi Warita⁸  Naoki Suzuki⁸  Rumiko Izumi⁸  Masaaki Kato⁸  Maki Tateyama⁸  Masashi Aoki⁸  Ken-ya Murata⁹  Tomoyoshi Kondo⁹  Ichizo Nishino^1,10 

[1] Department of Clinical Neuroscience, Institute of Biomedical Sciences, Tokushima University Graduate School;Department of Neurology and Geriatrics, Kagoshima University Graduate School of Medical and Dental Sciences;Department of Neurology, Graduate School of Medical Sciences, Kumamoto University;Department of Neurology, Kansai Medical University;Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry (NCNP);Department of Neurology, National Hospital Organization Sendai-Nishitaga National Hospital;Department of Neurology, Osaka City General Hospital;Department of Neurology, Tohoku University Graduate School of Medicine;Department of Neurology, Wakayama Medical University;Department of Neuromuscular Research, National Institute of Neuroscience;

关键词: Sporadic inclusion body myositis;    Multicenter survey;    Questionnaire;    Aging;    Muscle disease;   

DOI  :  10.1186/s13023-016-0524-x

来源: DOAJ

【 摘 要 】

Abstract Background Sporadic inclusion body myositis (sIBM) is the most prevalent acquired muscle disease in the elderly. sIBM is an intractable and progressive disease of unknown cause and without effective treatment. The etiology of sIBM is still unknown; however, genetic factors, aging, lifestyles, and environmental factors may be involved. The purpose of this study is to elucidate the cross-sectional profile of patients affected by sIBM in Japan. Methods We surveyed patient data for 146 cases diagnosed at a number of centers across Japan. We also issued a questionnaire for 67 patients and direct caregivers to further elucidate the natural history of the disease. Results The mean age at the onset was 63.4 ± 9.2 years. The mean length of time from the onset to diagnosis was 55.52 ± 49.72 months, suggesting that there is a difficulty in diagnosing this disease with long-term consequences because of late treatment. 73 % described the psychological/mental aspect of the disease. The most popular primary caregiver was the patient’s spouse and 57 % patients mentioned that they were having problems managing the finances. Conclusions Through these surveys, we described the cross-sectional profiles of sIBM in Japan. Many patients described psychological/mental and financial anxiety because of the aged profile of sIBM patients. The profiles of sIBM patients are similar to those in Western countries.

【 授权许可】

Unknown