TY - JOUR
T1 - The updated retrospective questionnaire study of sporadic inclusion body myositis in Japan
AU - Suzuki, Naoki
AU - Mori-Yoshimura, Madoka
AU - Yamashita, Satoshi
AU - Nakano, Satoshi
AU - Murata, Ken Ya
AU - Mori, Megumi
AU - Inamori, Yukie
AU - Matsui, Naoko
AU - Kimura, En
AU - Kusaka, Hirofumi
AU - Kondo, Tomoyoshi
AU - Ito, Hidefumi
AU - Higuchi, Itsuro
AU - Hashiguchi, Akihiro
AU - Nodera, Hiroyuki
AU - Kaji, Ryuji
AU - Tateyama, Maki
AU - Izumi, Rumiko
AU - Ono, Hiroya
AU - Kato, Masaaki
AU - Warita, Hitoshi
AU - Takahashi, Toshiaki
AU - Nishino, Ichizo
AU - Aoki, Masashi
N1 - Funding Information:
This study was supported by an Intramural Research Grant (26–7, 26–8) for Neurological and Psychiatric Disorders through the NCNP; a grant on Research on Rare and Intractable Diseases (H26-intractable disease 037 and 082) from the Ministry of Health, Labour and Welfare of Japan; the Ministry of Health, Labour and Welfare of Japan (H26-nanchitou (nan)-ippan-079); Health and Labour Sciences Research Grants for Comprehensive Research on Persons with Disabilities from Japan Agency for Medical Research and Development (15Adk0310043h0002); Grants-in-Aid for research on rare and intractable diseases; and Grant-in-Aid for Challenging Exploratory Research (26670436) from the Japanese Ministry of Education, Culture, Sports, Science and Technology.
Publisher Copyright:
© 2019 The Author(s).
PY - 2019/6/26
Y1 - 2019/6/26
N2 - Background: Sporadic inclusion body myositis (sIBM) is the most prevalent muscle disease in elderly people, affecting the daily activities. sIBM is progressive with unknown cause and without effective treatment. In 2015, sIBM was classified as an intractable disease by the Japanese government, and the treatment cost was partly covered by the government. This study aimed to examine the changes in the number of patients with sIBM over the last 10 years and to elucidate the cross-sectional profile of Japanese patients with sIBM. Methods: The number of sIBM patients was estimated through a reply-paid postcard questionnaire for attending physicians. Only patients diagnosed as "definite" or "probable" sIBM by clinical and biopsy sIBM criteria were included in this study (Lancet Neurol 6:620-631, 2007, Neuromuscul Disord 23:1044-1055, 2013). Additionally, a registered self-administered questionnaire was also sent to 106 patients who agreed to reply via their attending physician, between November 2016 and March 2017. Results: The number of patients diagnosed with sIBM for each 5-year period was 286 and 384 in 2011 and 2016, respectively. Inability to stand-up, cane-dependent gait, inability to open a plastic bottle, choking on food ingestion, and being wheelchair-bound should be included as sIBM milestones. Eight patients were positive for anti-hepatitis C virus antibody; three of them were administered interferon before sIBM onset. Steroids were administered to 33 patients (31.1%) and intravenous immunoglobulin to 46 patients (43.4%). From 2016 to 2017, total of 70 patients applied for the designated incurable disease medical expenses subsidy program. Although the treatment cost was partly covered by the government, many patients expressed psychological/mental and financial anxieties. Conclusions: We determined the cross-sectional profile of Japanese patients with sIBM. Continuous support and prospective surveys are warranted.
AB - Background: Sporadic inclusion body myositis (sIBM) is the most prevalent muscle disease in elderly people, affecting the daily activities. sIBM is progressive with unknown cause and without effective treatment. In 2015, sIBM was classified as an intractable disease by the Japanese government, and the treatment cost was partly covered by the government. This study aimed to examine the changes in the number of patients with sIBM over the last 10 years and to elucidate the cross-sectional profile of Japanese patients with sIBM. Methods: The number of sIBM patients was estimated through a reply-paid postcard questionnaire for attending physicians. Only patients diagnosed as "definite" or "probable" sIBM by clinical and biopsy sIBM criteria were included in this study (Lancet Neurol 6:620-631, 2007, Neuromuscul Disord 23:1044-1055, 2013). Additionally, a registered self-administered questionnaire was also sent to 106 patients who agreed to reply via their attending physician, between November 2016 and March 2017. Results: The number of patients diagnosed with sIBM for each 5-year period was 286 and 384 in 2011 and 2016, respectively. Inability to stand-up, cane-dependent gait, inability to open a plastic bottle, choking on food ingestion, and being wheelchair-bound should be included as sIBM milestones. Eight patients were positive for anti-hepatitis C virus antibody; three of them were administered interferon before sIBM onset. Steroids were administered to 33 patients (31.1%) and intravenous immunoglobulin to 46 patients (43.4%). From 2016 to 2017, total of 70 patients applied for the designated incurable disease medical expenses subsidy program. Although the treatment cost was partly covered by the government, many patients expressed psychological/mental and financial anxieties. Conclusions: We determined the cross-sectional profile of Japanese patients with sIBM. Continuous support and prospective surveys are warranted.
KW - Aging
KW - Multicenter survey
KW - Muscle diseasef
KW - Questionnaire
KW - Sporadic inclusion body myositis
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U2 - 10.1186/s13023-019-1122-5
DO - 10.1186/s13023-019-1122-5
M3 - Article
C2 - 31242950
AN - SCOPUS:85068889340
VL - 14
JO - Orphanet Journal of Rare Diseases
JF - Orphanet Journal of Rare Diseases
SN - 1750-1172
IS - 1
M1 - 155
ER -