Jordan Greco Anne-Sophie, Métrailler Jean-Claude, Dayer Eric
Département de médecine interne, CHCVs/Hôpital de Sierre.
Rev Med Suisse. 2007 Nov 21;3(134):2675-6, 2679-81.
The anti-synthetase syndrome is associated with inflammatory myopathy, interstitial lung disease, polyarthritis, Raynaud's phenomenon and typical skin lesions known as "mechanic's hands". Anti-Jo1 antibodies are frequently present. Interstitial lung disease is the most important feature, as it determines survival and the therapeutic attitude. We report here two cases of anti-synthetase syndrome with acute respiratory failure. Immunosuppressive treatment combining corticosteroids and cyclosporine lead to clinical improvement, regression of radiological lesions and improvement in pulmonary functions.
抗合成酶综合征与炎性肌病、间质性肺疾病、多关节炎、雷诺现象以及被称为“技工手”的典型皮肤病变相关。抗Jo1抗体常呈阳性。间质性肺疾病是最重要的特征,因为它决定了患者的生存率和治疗方案。我们在此报告两例伴有急性呼吸衰竭的抗合成酶综合征病例。联合使用皮质类固醇和环孢素的免疫抑制治疗使临床症状改善、放射学病变消退且肺功能得到改善。