摘要
抗合成酶综合症是一组密切相关的罕见疾病,临床表现为炎症性肌病、间质性肺疾病、关节炎、皮肤角化(机械手)和雷诺现象。抗合成酶综合症的病理生理机制尚不完全清楚,但遗传因素、病毒感染和药物的使用可能会起到一定的作用。某些抗合成酶抗体与各种临床表现和低负担的炎性肌病相关。与单纯炎性肌病比较,抗合成酶综合症患者的预后差比例更高,其主要原因是间质性肺疾病。未来的研究应进一步探讨抗合成酶综合征的发病机制,确定新的治疗靶标。抗合成酶综合症患者得癌症的风险是否增加,某些抗合成酶抗体是否与恶性肿瘤相关,这些都是今后中药的研究课题。
关键词: 抗合成酶抗体,抗合成酶综合征,自身免疫性疾病,癌症,糖皮质激素,炎症性肌病,间质性肺疾病,雷诺氏现象。
Current Medicinal Chemistry
Title:Antisynthetase Syndrome: A Review of Etiopathogenesis, Diagnosis and Management
Volume: 22 Issue: 16
Author(s): Aibek E. Mirrakhimov
Affiliation:
关键词: 抗合成酶抗体,抗合成酶综合征,自身免疫性疾病,癌症,糖皮质激素,炎症性肌病,间质性肺疾病,雷诺氏现象。
摘要: Antisynthetase syndrome is a group of closely related rare diseases which clinically manifest with inflammatory myopathies, interstitial lung disease, inflammatory arthritis, skin hyperkeratosis (mechanic’s hands) and Raynaud phenomenon. The pathophysiology of antisynthetase syndrome is not entirely understood, but genetic predisposition, viral infections and medication use may play a role. Certain antisynthetase antibodies are associated with various clinical presentations and a lower burden of inflammatory myopathies. Patients with antisynthetase syndrome have a worse prognosis than patients with pure inflammatory myopathies mainly because of interstitial lung disease. Future research should further investigate the pathogenesis of antisynthetase syndrome which could identify new therapeutic targets. It will be also important to study whether patients with AS are at increased risk of cancer and whether certain antisynthetase antibodies have any association with the risk of malignancy.
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Cite this article as:
Aibek E. Mirrakhimov , Antisynthetase Syndrome: A Review of Etiopathogenesis, Diagnosis and Management, Current Medicinal Chemistry 2015; 22 (16) . https://dx.doi.org/10.2174/0929867322666150514094935
DOI https://dx.doi.org/10.2174/0929867322666150514094935 |
Print ISSN 0929-8673 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-533X |
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