Thymectomy may not be associated with clinical improvement in MuSK myasthenia gravis
Thymectomy may not be associated with clinical improvement in MuSK myasthenia gravis
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DOI：
10.1002/mus.26404
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发表时间：
2019-04-01
期刊：
影响因子：
3.4
通讯作者：
Hehir, Michael K.
中科院分区：
文献类型：
--
作者：
Clifford, Katherine M.;Hobson-Webb, Lisa D.;Hehir, Michael K.
一项随机试验证明胸腺切除术对非胸腺瘤性乙酰胆碱受体（AChR）抗体阳性的重症肌无力（MG）有好处。未控制的观察和组织学研究表明胸腺切除术可能对抗肌肉特异性激酶(MuSK)-MG无效。方法：对麝香- mg患者的利妥昔单抗进行多中心、回顾性盲法评价胸腺切除术的治疗效果。结果：胸腺切除术（n = 26）组和非胸腺切除术（n = 29）组的基线特征相似，包括使用利妥昔单抗治疗（42%对45%）。在最后一次访问时，35%的胸腺切除术患者达到了主要终点，即美国重症肌无力基金会（MGFA）干预后状态（PIS）评分最小表现（MM）或更好，而对照组为55% （P = 0.17）。在控制MG、利妥昔单抗、强的松和静脉免疫球蛋白/血浆交换治疗的发病年龄后，胸腺切除术与良好临床结果的可能性不相关（优势比= 0.43,95%可信区间0.12-1.53,P = 0.19）。讨论：在这个多中心的麝香mg患者队列中，胸腺切除术与额外的临床改善无关。肌肉神经，2019
Introduction: A randomized trial demonstrated benefit from thymectomy in nonthymomatous acetylcholine receptor (AChR)-antibody positive myasthenia gravis (MG). Uncontrolled observational and histologic studies suggest thymectomy may not be efficacious in anti-muscle-specific kinase (MuSK)-MG. Methods: The therapeutic impact of thymectomy was evaluated from data collected for a multicenter, retrospective blinded review of rituximab in MuSK-MG. Results: Baseline characteristics were similar between thymectomy (n = 26) and nonthymectomy (n = 29) groups, including treatment with rituximab (42% vs. 45%). At last visit, 35% of thymectomy subjects reached the primary endpoint, a Myasthenia Gravis Foundation of America (MGFA) post-intervention status (PIS) score of minimal manifestations (MM) or better, compared with 55% of controls (P = 0.17). After controlling for age at onset of MG, rituximab, prednisone, and intravenous immunoglobulin/plasma exchange treatment, thymectomy was not associated with greater likelihood of favorable clinical outcome (odds ratio = 0.43, 95% confidence interval 0.12-1.53, P = 0.19). Discussion: Thymectomy was not associated with additional clinical improvement in this multicenter cohort of MuSK-MG patients. Muscle Nerve 59:404-410, 2019