Multicenter questionnaire survey for sporadic inclusion body myositis in Japan

Abstract

BackgroundSporadic 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.MethodsWe 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.ResultsThe 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.ConclusionsThrough 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.

The updated retrospective questionnaire study of sporadic inclusion body myositis in Japan

“…3a), as subjectively evaluated. This is consistent with the findings of our previous survey [18]. Further structured questionnaire in detail should be examined to analyze the severity of dementia.…”

“…Reply-paid postcard questionnaires were sent to the board-certified members of the Japanese Society of Neurology. In our previous study, the number such questionnaires sent was 4857 [18], whereas in the present study, 5500 were sent. The contents of the questionnaire are listed in Table 1.…”

“…In our previous study, we sent a total of 4857 questionnaires [18], whereas in the present study, 5500 questionnaires were sent. The number of responses were 1253 and 1316 in the previous and present studies (Table 4), indicating a reply rate of 25.8 and 23.9%, respectively.…”

“…Additionally, the respondents were asked to mention important disease milestones (Table 5). In the previous study, the most common initial symptom was the weakness of the proximal lower muscles, including the quadriceps femoris ( n = 117, 80%), followed by the weakness of finger flexors ( n = 9) and shoulder girdle muscle ( n = 5), muscle pain ( n = 3), general fatigue ( n = 3), and dysphagia ( n = 5) [18]. In the present study, we aimed to identify an array of milestones based on patients’ reports.…”

HLA-DRB1 allele and autoantibody profiles in Japanese patients with inclusion body myositis

“…However, it is noted that DRB1 � 0301 is rarely detectable in the Japanese population. Although the clinical profiles of Japanese patients with IBM are similar to those of Caucasian patients [13], the immunogenetic background and autoantibody profiles have not been fully elucidated.…”