Impact of treatment on prognosis of inflammatory myopathy-associated cardiomyopathy / 中华全科医师杂志

ABSTRACT

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 death1/14 vs.8/15,P=0.014;cardiac death0 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 x2=6.001,HR=7.58,P=0.014),as well as between patients with β receptor blockers and controls (Log Rank x2=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,and β receptor blockers were worthy to be considered.