难治性重症肌无力-临床概况,合并症和对利妥昔单抗的反应

Q1 Medicine GMS German Medical Science Pub Date : 2016-10-13 DOI:10.3205/000239
Sreenivasa Rao Sudulagunta, Mona Sepehrar, Mahesh Babu Sodalagunta, Aravinda Settikere Nataraju, Shiva Kumar Bangalore Raja, D. Sathyanarayana, Siddharth Gummadi, Hema Burra
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引用次数: 32

摘要

重症肌无力是一种以疲劳性肌肉无力为特征的抗体介导的自身免疫性神经肌肉疾病。一部分重症肌无力患者由于对常规治疗无反应而被归类为难治性。本回顾性研究旨在评估MG患者的临床概况、流行病学、实验室和特征,以及利妥昔单抗的治疗模式和并发症。方法:纳入2008年1月至2016年1月期间因MG入院或门诊就诊的重症肌无力患者(既往病历)的资料。512例患者符合重症肌无力的临床及诊断标准,其中76例患者符合难治性重症肌无力的诊断确定性并进行评价。结果:76例难治性MG患者中,53例(69.73%)患者满足所有三个定义标准。难治性MG组的中位发病年龄为36岁,范围为27-53岁。本组25例患者(32.89%)年龄在21 ~ 30岁。非难治性MG患者8例(2.06%)、难治性MG患者36例(47.36%)出现抗麝香抗体阳性。平均HbA1C为8.6±2.33。在利妥昔单抗治疗第三个周期后,泼尼松给药剂量平均减少59.7% (p= 3.3x10-8)至94.6% (p= 2.2x10-14)。结论:难治性MG患者多为女性,发病年龄早,有抗麝香抗体和胸腺瘤。难治性MG患者的糖尿病和血脂异常患病率较高,控制较差(HbA1C≤8%),可能是由于类固醇使用增加所致。利妥昔单抗治疗难治性MG非常有效,不良反应低。
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Refractory myasthenia gravis – clinical profile, comorbidities and response to rituximab
Introduction: Myasthenia gravis (MG) is an antibody mediated autoimmune neuromuscular disorder characterized by fatigable muscle weakness. A proportion of myasthenia gravis patients are classified as refractory due to non responsiveness to conventional treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and features of patients with MG and mode of management using rituximab and complications. Methods: Data of myasthenia gravis patients admitted or presented to outpatient department (previous medical records) with MG between January 2008 and January 2016 were included. A total of 512 patients fulfilled the clinical and diagnostic criteria of myasthenia gravis of which 76 patients met the diagnostic certainty for refractory myasthenia gravis and were evaluated. Results: Out of 76 refractory MG patients, 53 (69.73%) patients fulfilled all the three defined criteria. The median age of onset of the refractory MG group was 36 years with a range of 27–53 years. In our study 25 patients (32.89%) belonged to the age group of 21–30 years. Anti-MuSK antibodies were positive in 8 non-refractory MG patients (2.06%) and 36 refractory MG patients (47.36%). Mean HbA1C was found to be 8.6±2.33. The dose of administered prednisone decreased by a mean of 59.7% (p=3.3x10–8) to 94.6% (p=2.2x10–14) after the third cycle of rituximab treatment. Conclusion: The refractory MG patients are most commonly female with an early age of onset, anti-MuSK antibodies, and thymomas. Refractory MG patients have higher prevalence and poor control (HbA1C >8%) of diabetes mellitus and dyslipidemia probably due to increased steroid usage. Rituximab is very efficient in treatment of refractory MG with adverse effects being low.
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来源期刊
GMS German Medical Science
GMS German Medical Science Medicine-Medicine (all)
CiteScore
6.30
自引率
0.00%
发文量
10
审稿时长
11 weeks
期刊最新文献
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