Suzuki, Naoki Tohoku University
Mori-Yoshimura, Madoka National Center of Neurology and Psychiatry
Yamashita, Satoshi Kumamoto University
Nakano, Satoshi Osaka City General Hospital
Murata, Ken-ya Wakayama Medical University
Inamori, Yukie Kagoshima University
Kimura, En Kumamoto University
Kusaka, Hirofumi Kansai Medical University
Kondo, Tomoyoshi Wakayama Medical University
Higuchi, Itsuro Kagoshima University
Tateyama, Maki Tohoku University
Izumi, Rumiko Tohoku University
Ono, Hiroya Tohoku University
Kato, Masaaki Tohoku University
Warita, Hitoshi Tohoku University
Takahashi, Toshiaki National Hospital Organization Sendai-Nishitaga National Hospital
Nishino, Ichizo National Institute of Neuroscience|National Center of Neurology and Psychiatry
Aoki, Masashi Tohoku University
Sporadic inclusion body myositis
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.
Orphanet Journal of Rare Diseases
BioMed Central|Springer Nature
© 2016 The Author(s). Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
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