Experience with programmed steroid treatment with thymectomy in nonthymomatous myasthenia gravis
Experience with programmed steroid treatment with thymectomy in nonthymomatous myasthenia gravis
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DOI：
10.1016/j.athoracsur.2003.10.039
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发表时间：
2004-05-01
影响因子：
4.6
通讯作者：
Sohara, Y
中科院分区：
文献类型：
--
作者：
Endo, S;Yamaguchi, T;Sohara, Y
背景资料。胸腺切除在重症肌无力治疗中的益处是公认的，但围手术期的病程可能会波动。本研究的目的是评估胸腺切除加剂量递增的类固醇治疗非胸腺性肌无力的可行性和临床益处。我们回顾了自1976-2000年间，69例重症肌无力患者接受胸骨胸腺切除并扩大前纵隔清扫术后的随访记录。程序化治疗组中48例在围手术期进行了剂量递增和递减激素治疗，其中17例为眼肌无力，31例为全身性重症肌无力。比较两组患者的临床疗效和临床缓解情况，比较两组患者在围手术期期间偶尔用药的21例患者的临床疗效和临床缓解。程序化治疗组术后未发生呼吸衰竭和肌无力危象，而偶发治疗组有6例发生。程序化治疗组(平均随访6.4年)3年、5年、10年缓解率分别为30%、38%、46%；间歇性治疗组(平均随访9.6年)3年、5年、10年缓解率分别为25%、25%、45%。程序化类固醇治疗非胸腺瘤性重症肌无力患者是可行的，并且在围手术期出现波动症状时提供临床益处。
Background. The benefit of thymectomy in myasthenia gravis management is recognized but the perioperative course can fluctuate. The goal of this study was to assess the feasibility and clinical benefit of dose-escalated steroid therapy with thymectomy for nonthymornatous myasthenia gravis.Methods. We reviewed the records of 69 myasthenia gravis patients who were followed up after undergoing transsternal thymectomy with extended anterior mediastinal dissection in our hospital between 1976-2000. Forty-eight patients in the programmed treatment group who had dose-escalated and de-escalated steroid therapy during the perioperative period comprised 17 patients with ocular myasthenia gravis and 31 patients with generalized myasthenia gravis. Clinical benefits and clinical remission, which was diagnosed when the patients were symptom-free without medications for at least 1 year, were compared with those of 21 patients in the occasional treatment group who received medications occasionally over the perioperative period.Results. Postoperative respiratory failure and myasthenic crisis did not occur in the programmed treatment group but did occur in 6 patients in the occasional treatment group. Remission rates in the programmed treatment group (mean follow-up, 6.4 years) were 30% at 3 years, 38% at 5 years, and 46% at 10 years; rates in the occasional treatment group (mean follow-up, 9.6 years) were 25% at 3 years, 25% at 5 years, and 45% at 10 years.Conclusions. Programmed steroid therapy in patients with nonthymomatous myasthenia gravis is feasible and it provides clinical benefit when fluctuating symptoms occur during the perioperative period.