Ponseti José M, Gamez Josep, Azem Jamal, López-Cano Manuel, Vilallonga Ramón, Armengol Manuel
Unit of Myasthenia Gravis, Department of Surgery, Hospital General Universitari Vall d'Hebron, Passeig Vall d'Hebron 119-129, E-08035 Barcelona, Spain.
Ann N Y Acad Sci. 2008;1132:254-63. doi: 10.1196/annals.1405.000. Epub 2007 Dec 20.
Tacrolimus is a macrolide T cell immunomodulator that is used in myasthenia gravis (MG) patients to affect muscle contraction (ryanodine receptor by modulating intracellular calcium-release channels and increasing muscular strength), glucocorticoid receptors (increasing intracellular concentration of steroids and blocking the steroid export mechanism), and an increase in T cell apoptosis. In this study, we report the results of low-dose tacrolimus (0.1 mg/kg/day) treatment in 212 MG patients. There were 110 thymectomized, cyclosporine- and prednisone-dependent patients; 68 thymectomized patients who started tacrolimus early postoperatively (24 h after operation); and 34 patients over 60 years old with nonthymomatous generalized MG or in whom thymectomy was contraindicated. The mean follow-up time was 49.3 +/- 18.1 months. Muscular strength showed an increase of 23% after 1 month of treatment and 29% at the end of the study. The acetylcholine receptor antibodies decreased significantly from a mean of 33.5 nmol/L at base line to 7.8 nmol/L at the final visit. In the thymectomy group with combined prednisone and tacrolimus stratified by histology of the thymus, the mean probability to attain complete stable remission at 5 years was 80.8% in patients with hyperplasia, 48.1% in thymic involution, and 9.3% in patients with thymoma. In 4.9% of patients, tacrolimus was withdrawn because of major adverse effects. Our results suggest that a low dose of tacrolimus is effective for MG and could be included to the armamentarium for this autoimmune disease. The present results should be interpreted considering the limitations of a retrospective clinical study. Confirmation of these results in randomized studies is desirable.
他克莫司是一种大环内酯类T细胞免疫调节剂,用于重症肌无力(MG)患者,以影响肌肉收缩(通过调节细胞内钙释放通道和增加肌肉力量作用于兰尼碱受体)、糖皮质激素受体(增加细胞内类固醇浓度并阻断类固醇输出机制),并增加T细胞凋亡。在本研究中,我们报告了212例MG患者接受低剂量他克莫司(0.1mg/kg/天)治疗的结果。其中有110例接受过胸腺切除术、依赖环孢素和泼尼松的患者;68例术后早期(术后24小时)开始使用他克莫司的胸腺切除患者;以及34例60岁以上患有非胸腺瘤性全身型MG或胸腺切除术禁忌的患者。平均随访时间为49.3±18.1个月。治疗1个月后肌肉力量增加了23%,研究结束时增加了29%。乙酰胆碱受体抗体从基线时的平均33.5nmol/L显著降至最后一次随访时的7.8nmol/L。在胸腺切除联合泼尼松和他克莫司治疗组中,根据胸腺组织学分层,5年时达到完全稳定缓解的平均概率在增生患者中为80.8%,胸腺萎缩患者中为48.1%,胸腺瘤患者中为9.3%。4.9%的患者因严重不良反应停用他克莫司。我们的结果表明,低剂量他克莫司对MG有效,可纳入这种自身免疫性疾病的治疗手段。应考虑到回顾性临床研究的局限性来解释目前的结果。希望在随机研究中证实这些结果。