Multicenter questionnaire survey for sporadic inclusion body myositis in Japan.

Multicenter questionnaire survey for sporadic inclusion body myositis in Japan.
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DOI:
10.1186/s13023-016-0524-x
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发表时间:
2016-11-08
影响因子:
3.7
通讯作者:
Aoki M
Aoki M
中科院分区:
医学2区
文献类型:
--
作者:
Suzuki N;Mori-Yoshimura M;Yamashita S;Nakano S;Murata KY;Inamori Y;Matsui N;Kimura E;Kusaka H;Kondo T;Higuchi I;Kaji R;Tateyama M;Izumi R;Ono H;Kato M;Warita H;Takahashi T;Nishino I;Aoki M

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散发性包涵体肌炎(sIBM)是老年人中最常见的获得性肌肉疾病。sIBM是一种原因不明的难治性进行性疾病,目前尚无有效的治疗方法。sIBM的病因尚不清楚;然而,可能涉及遗传因素、衰老、生活方式和环境因素。本研究的目的是阐明日本sIBM患者的横截面特征。我们调查了日本各地多家中心诊断的146例患者的数据。我们还对67名患者和直接护理人员进行了问卷调查,以进一步阐明疾病的自然史。发病时的平均年龄为63.4 ± 9.2岁。从发病到确诊的平均时间为55.52 ± 49.72个月,这表明由于治疗较晚,很难诊断这种疾病并造成长期后果。73%描述了疾病的心理/精神方面。最受欢迎的主要照顾者是病人的配偶,57%的病人提到他们在管理财务方面有问题。通过这些调查,我们描述了日本sIBM的横截面轮廓。由于sIBM患者的年龄特征,许多患者描述了心理/精神和经济焦虑。sIBM患者的特征与西方国家相似。
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. 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. 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. 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.
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