目的:报道2 例双重滤过血浆置换(double filtration plasmapheresis,DFPP)联合口服小剂量醋酸泼尼松治疗全身型重症肌无力(myasthenia gravis,MG)的病例,旨在为 MG 临床治疗提供思路。

方法:报道2 例全身型 MG 患者的临床症状、辅助检查结果、诊疗经过以及预后,并结合相关文献复习,讨论不同治疗方法的作用。

结果:病例1以“胸闷、气促3月余,加重伴双眼睑下垂、饮水呛咳1月”为主要临床表现,入院后完善相关检查，疲劳试验阳性，新斯的明试验阳性，四肢肌力4级，血清抗乙酰胆碱受体(acetylcholine receptor,AchR)抗体阳性,重症肌无力评分(Quantitative MG Score,QMGS)为19 分,MG日常生活质量(MG-activities of Daily Living Profile,MG-ADL)为 13 分。病例 2因“吞咽乏力1月,加重伴四肢乏力1周余”入院诊治,查四肢肌力5级,洼田饮水试验3级,疲劳试验阳性。QMGS为6分,MG-ADL为11分,抗AchR抗体>80nmol。综合患者的临床表现和辅助检查结果,2 例均诊断为 MG 中度全身型,急性加重期。2 例患者均采用 DFPP 联合醋酸泼尼松口服治疗，患者临床症状均改善。治疗后2例患者 QMGS 评分分别为7分和0分,MG-ADL分别为 5 分和 0分。

结论:本研究采取 DFPP 联合口服小剂量醋酸泼尼松治疗的方式,不仅能够充分发挥药物之间的协同作用,有效改善 MG 患者的临床症状,并且可预防长时间使用激素而导致的不良反应。所以该病例治疗方法对 MG 的临床治疗有一定的提示意义。

Objective: Two cases of double filtration plasmapheresis (DFPp) combined with oral low-dose prednisone acetate were reported for the treatment of systemic myasthenia gravis (MG), aiming to provide ideas for the clinical treatment of MG.

Methods: The clinical symptoms, auxiliary examination results, diagnosis and treatment process and prognosis of 2 patients with systemic MG were reported. The effects of different treatment methods were analyzed by reviewing relevant literature.

Results: In case 1, the main clinical manifestations were "chest tightness and shortess of breath for more than 3 months, aggravated by drooping eyelids and coughing due to drinking water for 1 month". After admission, relevant examinations were improved, the fatigue test and neostigmine test were positive, musce strength of limbs was grade 4,serum anti acetylcholine receptor (AchR) antibody was positive, and myasthenia gravis score (QMGS) was 19 points.MG-activities of daily living profile(MG-ADL) was 13 points. Case 2 was admitted for diagnosis and treatment due to "dysphagia for one month, aggravated by weakness in the limbs for more than a week". Muscle strength in the limbs was found to be level 5, and the Wada drinking water test was level 3. The fatigue test was positive.QMGS was 6 points, MG-ADL was 11 points, blood anti-AchR antibody >80 nmol/L.Combined with the clinical manifestations and auxiliary examination results, 2 cases were diagnosed as MG moderate systemic type, acute exacerbation period. After treatment with DFPP combined with oral prednisone acetate, the clinical symptoms of 2 patients were improved. After treatment, the QMGS scores of the 2 patients were 7 and 0, respectively,and the MG-ADL scores were 5 and 0,respectively.

Conclusion:In this study, DFPPcombined with oral low-dose prednisone acetate was used for treatment, which not only gave full play to the synergistic effect between drugs,effectively improved the clinical symptoms of MG patients, and prevented the adverse reactions caused by long-term use of corticosteroids. Therefore, the treatment method of this case has certain suggestive significance for the clinical treatment of MG.