Multicenter questionnaire survey for sporadic inclusion body myositis in Japan
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, lifestyle...
| Main Authors: | , , , , , , , , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2016-11-01
|
| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13023-016-0524-x |
| _version_ | 1819203962529644544 |
|---|---|
| author | Naoki Suzuki Madoka Mori-Yoshimura Satoshi Yamashita Satoshi Nakano Ken-ya Murata Yukie Inamori Naoko Matsui En Kimura Hirofumi Kusaka Tomoyoshi Kondo Itsuro Higuchi Ryuji Kaji Maki Tateyama Rumiko Izumi Hiroya Ono Masaaki Kato Hitoshi Warita Toshiaki Takahashi Ichizo Nishino Masashi Aoki |
| author_facet | Naoki Suzuki Madoka Mori-Yoshimura Satoshi Yamashita Satoshi Nakano Ken-ya Murata Yukie Inamori Naoko Matsui En Kimura Hirofumi Kusaka Tomoyoshi Kondo Itsuro Higuchi Ryuji Kaji Maki Tateyama Rumiko Izumi Hiroya Ono Masaaki Kato Hitoshi Warita Toshiaki Takahashi Ichizo Nishino Masashi Aoki |
| author_sort | Naoki Suzuki |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-23T04:28:15Z |
| format | Article |
| id | doaj.art-ccf1c87722e743dcae8206450e0f9648 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-23T04:28:15Z |
| publishDate | 2016-11-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-ccf1c87722e743dcae8206450e0f96482022-12-21T18:00:06ZengBMCOrphanet Journal of Rare Diseases1750-11722016-11-011111710.1186/s13023-016-0524-xMulticenter questionnaire survey for sporadic inclusion body myositis in JapanNaoki Suzuki0Madoka Mori-Yoshimura1Satoshi Yamashita2Satoshi Nakano3Ken-ya Murata4Yukie Inamori5Naoko Matsui6En Kimura7Hirofumi Kusaka8Tomoyoshi Kondo9Itsuro Higuchi10Ryuji Kaji11Maki Tateyama12Rumiko Izumi13Hiroya Ono14Masaaki Kato15Hitoshi Warita16Toshiaki Takahashi17Ichizo Nishino18Masashi Aoki19Department of Neurology, Tohoku University Graduate School of MedicineDepartment of Neurology, National Center Hospital, National Center of Neurology and Psychiatry (NCNP)Department of Neurology, Graduate School of Medical Sciences, Kumamoto UniversityDepartment of Neurology, Osaka City General HospitalDepartment of Neurology, Wakayama Medical UniversityDepartment of Neurology and Geriatrics, Kagoshima University Graduate School of Medical and Dental SciencesDepartment of Clinical Neuroscience, Institute of Biomedical Sciences, Tokushima University Graduate SchoolDepartment of Neurology, Graduate School of Medical Sciences, Kumamoto UniversityDepartment of Neurology, Kansai Medical UniversityDepartment of Neurology, Wakayama Medical UniversityDepartment of Neurology and Geriatrics, Kagoshima University Graduate School of Medical and Dental SciencesDepartment of Clinical Neuroscience, Institute of Biomedical Sciences, Tokushima University Graduate SchoolDepartment of Neurology, Tohoku University Graduate School of MedicineDepartment of Neurology, Tohoku University Graduate School of MedicineDepartment of Neurology, Tohoku University Graduate School of MedicineDepartment of Neurology, Tohoku University Graduate School of MedicineDepartment of Neurology, Tohoku University Graduate School of MedicineDepartment of Neurology, National Hospital Organization Sendai-Nishitaga National HospitalDepartment of Neuromuscular Research, National Institute of NeuroscienceDepartment of Neurology, Tohoku University Graduate School of MedicineAbstract 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.http://link.springer.com/article/10.1186/s13023-016-0524-xSporadic inclusion body myositisMulticenter surveyQuestionnaireAgingMuscle disease |
| spellingShingle | Naoki Suzuki Madoka Mori-Yoshimura Satoshi Yamashita Satoshi Nakano Ken-ya Murata Yukie Inamori Naoko Matsui En Kimura Hirofumi Kusaka Tomoyoshi Kondo Itsuro Higuchi Ryuji Kaji Maki Tateyama Rumiko Izumi Hiroya Ono Masaaki Kato Hitoshi Warita Toshiaki Takahashi Ichizo Nishino Masashi Aoki Multicenter questionnaire survey for sporadic inclusion body myositis in Japan Orphanet Journal of Rare Diseases Sporadic inclusion body myositis Multicenter survey Questionnaire Aging Muscle disease |
| title | Multicenter questionnaire survey for sporadic inclusion body myositis in Japan |
| title_full | Multicenter questionnaire survey for sporadic inclusion body myositis in Japan |
| title_fullStr | Multicenter questionnaire survey for sporadic inclusion body myositis in Japan |
| title_full_unstemmed | Multicenter questionnaire survey for sporadic inclusion body myositis in Japan |
| title_short | Multicenter questionnaire survey for sporadic inclusion body myositis in Japan |
| title_sort | multicenter questionnaire survey for sporadic inclusion body myositis in japan |
| topic | Sporadic inclusion body myositis Multicenter survey Questionnaire Aging Muscle disease |
| url | http://link.springer.com/article/10.1186/s13023-016-0524-x |
| work_keys_str_mv | AT naokisuzuki multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT madokamoriyoshimura multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT satoshiyamashita multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT satoshinakano multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT kenyamurata multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT yukieinamori multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT naokomatsui multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT enkimura multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT hirofumikusaka multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT tomoyoshikondo multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT itsurohiguchi multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT ryujikaji multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT makitateyama multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT rumikoizumi multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT hiroyaono multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT masaakikato multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT hitoshiwarita multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT toshiakitakahashi multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT ichizonishino multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan AT masashiaoki multicenterquestionnairesurveyforsporadicinclusionbodymyositisinjapan |