炎性肌病合并心肌病患者的治疗策略对预后的影响

Impact of treatment on prognosis of inflammatory myopathy-associated cardiomyopathy

目的观察炎性肌病合并心肌病患者的治疗策略及对预后的影响.方法回顾北京协和医院1999-2016年住院诊治的29例炎性肌病合并心肌病的初治患者,记录治疗方案,随访终点事件.结果29例患者,包括多发性肌炎11例,皮肌炎8例,重叠综合征8例,非特异性肌炎2例.所有患者起始均服用足量激素(泼尼松1~2 mg·kg^-1-d^-1).7例静脉输注免疫球蛋白,12例曾予激素冲击治疗.使用甲氨蝶呤(MTX)16例,环磷酰胺(CTX)15例,环孢素A(CsA)6例,联用2种免疫抑制剂(MTX联合CTX或CsA)11例.平均随访4.8年(范围2个月~15年),死亡14例,其中因心力衰竭死亡或心源性猝死9例.心脏事件死亡组患者均未使用免疫球蛋白,而对照组使用比例为7/20(P=0.05);心脏事件死亡组患者较对照组激素冲击治疗比例较低(1/9比11/20,P=0.043).全因死亡及心脏事件死亡组较对照组更少选择MTX联合CTX或CsA的免疫抑制治疗方案(全因死亡:1/14比8/15;心脏死亡:0比9/20,均P<0.05).Kaplan-Meier生存分析显示,MTX联合CTX或CsA治疗与对照组(LogRankx^2=6.001,HR=7.58,P=0.014)、β受体阻滞剂与对照组的炎性肌病心肌病患者的生存率差异均有统计学意义(Log Rank x^2=4.589,HR=2.95,P=0.032).结论合并心肌病的炎性肌病患者治疗策略应兼顾控制原发病情和改善心脏重构,在足量激素的基础上,积极考虑激素冲击治疗、IVIG及MTX+CTX/CsA的联合免疫抑制剂治疗.

Objective To observe the treatment of inflammatory myopathy-associatied cardiomyopathy and its impact on prognosis of disease.Methods In this single-center retrospective study,29 cases of inflammatory myopathy-associated cardiomyopathy were collected in Peking Union Medical College Hospital from 1999 to 2016.The clinical data and adverse events during follow up were documented.Among 29 patients there were 11 cases of polymyositis,8 cases of dermatomyositis,8 cases of overlap syndrome and 2 cases of nonspecific myositis.All the patients started with sufficiene prednisone(1-2 mg·kg^-1·d^-1).7 cases received intravenous immune globulin,while 12 cases were prescribed with steroid pulse therapy,16 cases with methotrexate,15 cases with cyclophosphamide,6 cases with cyclosporine A,while 11 cases with combination of immune suppressors(methotrexate plus cyclophosphamide or cyclosporine A).After a median follow up of 4.8 years(2 month to 15 years),14 cases died including 9 of cardiac death.Patients with cardiac deaths had lower usage percentages of intravenous gamma globulin(0 vs.7/20,P=0.05)and steroid pulse therapy(1/9 vs.11/20,P=0.043)than controls.Comparing with controls,patients in the group of adverse events were more prone to choose methotrexate alone rather than combination of immune suppressors(all-cause death:1/14 vs.8/15,P=0.014;cardiac death:0 vs.9/20,P=0.027).Kaplan-Meier survival analysis showed significant difference of survival rates between patients with combination of immune suppressors and controls(Log Rank x^2=6.001,HR=7.58,P=0.014),as well as between patients withβreceptor blockers and controls(Log Rank x^2=4.589,HR=2.95,P=0.032).Conclusions We recommend a management strategy that emphasize both primary disease controlling and cardiac remodeling improvement in patients with inflammatory myopathy-associated cardiomyopathy.On the basis of sufficient glucocorticoids,intravenous gamma globulin,steroid pulse therapy,combination of methotrexate plus other immune suppressants,andβreceptor blockers were worthy to be considered.