| H01604 | |
| H番号 | H01604 |
| 名称 | 皮膚筋炎/多発性筋炎 |
| 概要 | Polymyositis (PM) and dermatomyositis (DM) are the two major forms of inflammatory muscle diseases. PM and DM, along with sporadic inclusion-body myositis (sIBM), belong to the heterogeneous group of the idiopathic inflammatory myopathies (IIMs), which are characterized by weakness and chronic inflammation of skeletal muscle. PM and DM differ in their clinical features, histopathology, response to treatment, and prognosis. Although their clinical pictures differ, they both present with symmetrical, proximal muscle weakness. PM is a term that was used traditionally to denote all IIMs that were not DM or sIBM, but it is now a controversial entity with questionable specificity. Traditionally, PM is described as presenting with weakness of the proximal muscles that evolves over weeks to months and affects adults, but rarely children. DM typically includes subacute progressive proximal muscle weakness and a skin rash. The disease mechanisms of PM and DM that cause muscle damage and dysfunction are not fully understood. However, because of the association with other autoimmune diseases, the presence of autoantibodies, and response to immunosuppressive medication, they are believed to be autoimmune in origin. Recent studies have highlighted the importance of the innate immune system and non-immune mechanisms and described novel adaptive immune-based pathways in the pathogenesis of PM and DM. Treatment of inflammatory myopathies is generally empirical. Corticosteroids still remain the agents of choice for the initial treatment, but their use is limited by the high frequency of side effects. In addition, as a substantial number of patients do not respond to glucocorticoids alone, additional agents such as immunosuppressants, immunomodulators, and more recently, biologics are commonly used in clinical practice. |
| カテゴリ | 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 | デキサメタゾン [DR:D00292] デキサメタゾンリン酸エステルナトリウム [DR:D00975] ベタメタゾンリン酸エステルナトリウム [DR:D00972] トリアムシノロンアセトニド [DR:D00983] タクロリムス水和物 [DR:D00107] ヒドロコルチゾン [DR:D00088] トリアムシノロン [DR:D00385] ベタメタゾン [DR:D00244] プレドニゾロン [DR:D00472] プレドニゾロンコハク酸エステルナトリウム [DR:D01239] メチルプレドニゾロン [DR:D00407] メチルプレドニゾロンコハク酸エステルナトリウム [DR:D00751] メチルプレドニゾロン酢酸エステル [DR:D00979] アザチオプリン [DR:D00238] シクロホスファミド水和物 [DR:D00287] ポリエチレングリコール処理人免疫グロブリン [DR:D08783] |
| コメント | Autoantigen: HARS [HSA:3035] [KO:K01892] TARS [HSA:6897] [KO:K01868] AARS [HSA:16] [KO:K01872] GARS [HSA:2617] [KO:K01880] IARS [HSA:3376] [KO:K01870] NARS [HSA:4677] [KO:K01893] FARSA [HSA:2193] [KO:K01889] FARSB [HSA:10056] [KO:K01890] IFIH1 [HSA:64135] [KO:K12647] TRIM33 [HSA:51592] [KO:K08883] MORC3 [HSA:23515] SAE1 [HSA:10055] [KO:K10684] SAE2 [HSA:10054] [KO:K10685] |
| リンク | ICD-11: 4A41.0 4A41.1 ICD-10: M33 MeSH: D017285 D003882 |
| 文献 | PMID:26299472 著者 Vermaak E, Tansley SL, McHugh NJ タイトル The evidence for immunotherapy in dermatomyositis and polymyositis: a systematic review. 雑誌 Clin Rheumatol 34:2089-95 (2015) DOI:10.1007/s10067-015-3059-y PMID:26175404 著者 Lahouti AH, Christopher-Stine L タイトル Polymyositis and dermatomyositis: novel insights into the pathogenesis and potential therapeutic targets. 雑誌 Discov Med 19:463-70 (2015) PMID:23758833 著者 Rayavarapu S, Coley W, Kinder TB, Nagaraju K タイトル Idiopathic inflammatory myopathies: pathogenic mechanisms of muscle weakness. 雑誌 Skelet Muscle 3:13 (2013) DOI:10.1186/2044-5040-3-13 PMID:25641317 著者 Findlay AR, Goyal NA, Mozaffar T タイトル An overview of polymyositis and dermatomyositis. 雑誌 Muscle Nerve 51:638-56 (2015) DOI:10.1002/mus.24566 |