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.