| H01505 | |
| H番号 | H01505 |
| 名称 | 封入体筋炎 |
| 概要 | Inclusion body myositis (IBM) is the most frequent acquired myopathy after age 45. It is distinguished from other inflammatory myopathies by its selective pattern of muscle involvement and slowly progressive course, and by the combination of inflammatory and degenerative muscle pathology and multi-protein deposits in muscle tissue. It typically presents with chronic insidious proximal leg and/or distal arm asymmetric muscle weakness leading to recurrent falls and loss of dexterity. Creatine kinase (CK) is elevated in IBM and needle electromyography (EMG) mostly shows a chronic irritative myopathy. Muscle histopathology demonstrates endomysial inflammatory exudates surrounding and invading non-necrotic muscle fibers often times accompanied by rimmed vacuoles and protein deposits. IBM is refractory to all known immunosuppressive therapies. It has been shown in small short-term trials that individualised exercise programs can lead to improvement or maintenance of muscle strength and aerobic capacity. |
| カテゴリ | 神経系疾患; 筋骨格疾患 |
| ネットワーク | - |
| 病因遺伝子 | - |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | - |
| コメント | - |
| リンク | ICD-11: 4A41.20 ICD-10: G72.4 MeSH: D018979 |
| 文献 | PMID:25037082 著者 Dimachkie MM, Barohn RJ タイトル Inclusion body myositis. 雑誌 Neurol Clin 32:629-46, vii (2014) DOI:10.1016/j.ncl.2014.04.001 PMID:26778717 著者 Needham M, Mastaglia FL タイトル Sporadic inclusion body myositis: A review of recent clinical advances and current approaches to diagnosis and treatment. 雑誌 Clin Neurophysiol 127:1764-73 (2016) DOI:10.1016/j.clinph.2015.12.011 PMID:26335925 著者 Gang Q, Bettencourt C, Houlden H, Hanna MG, Machado PM タイトル Genetic advances in sporadic inclusion body myositis. 雑誌 Curr Opin Rheumatol 27:586-94 (2015) DOI:10.1097/BOR.0000000000000213 |