Sporadic Inclusion Body Myositis Manifesting as Isolated Muscle Weakness of the Finger Flexors Three Years after Disease Onset

Suwa, Yuichi; Suzuki, Naoki; Soga, Takashi; Harada, Ryuhei; Shibui, Aya; Kuroda, Hiroshi; Izumi, Rumiko; Tateyama, Masao; Nakashima, Ichiro; Sonoo, Masahiro; Akiyama, Masashi

doi:10.2169/internalmedicine.55.7285

Cited by 4 publications

(4 citation statements)

References 11 publications

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“…On the other hand, paying greater attention to dysphagia and wheelchair requirement should be the focus of the latter 5 years. Additionally, a fraction of the patients were observed to initially have partial or single sIBM symptom (e.g., dysphagia or inability to flex fingers), which may remain isolated for several years [15, 16]. No correlation was found between modified IBMFRS and the parameters examined (data not shown).…”

Section: Discussionmentioning

confidence: 99%

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

Suzuki

Mori‐Yoshimura

Yamashita

et al. 2019

Orphanet J Rare Dis

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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.

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Section: Discussionmentioning

confidence: 99%

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

Suzuki

Mori‐Yoshimura

Yamashita

et al. 2019

Orphanet J Rare Dis

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“…Recent studies have reported some clinical benefits of alemtuzumab, a monoclonal antibody directed at CD52 that depletes lymphocytes, in IBM patients. IBM is more frequent in males and is usually associated with asymmetric muscle weakness [2] . Symptoms frequently involve the finger flexors and knee extensors, and dysphasia is present in 40% of patients.…”

Section: Discussionmentioning

confidence: 99%

“…In this patient, the most prominent symptom was facial paresis. To our knowledge, there is only one other case in the literature of IBM with prominent facial involvement [1,2] . IBM is an inflammatory myopathy mediated by cytotoxic CD8+ T cells that clonally expand in situ and invade MHC-I-expressing muscle fibres.…”

Section: Discussionmentioning

confidence: 99%

Inclusion Body Myositis Treated with Alemtuzumab

Sá

Costelha

Marinho

2019

European Journal of Case Reports in Internal Medicine

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Inclusion body myositis (IBM) is a chronic inflammatory myopathy with a progressive course. It is more common in the later years of life and usually presents with limb weakness. We present the case of a patient who developed proximal weakness in the lower limbs and, four years later, facial asymmetry. Blood analysis revealed high lactate dehydrogenase and creatinine kinase values. The diagnosis was obtained through muscle biopsy which met the histological criteria for IBM. The patient started treatment with alemtuzumab, leading to stabilisation of the symptoms in two years.

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“…Some of the patients in our study visited several physicians and the average time from date of onset to diagnosis was 55.52 months. Some patients show isolated weakness of finger flexors 3 years after onset but were diagnosed with sIBM in muscle biopsy 6 years after onset [10]. Some patients underwent repeat biopsy of the muscle because of the lack of findings related to rimmed vacuoles or other pathological markers.…”

Section: Discussionmentioning

confidence: 99%