Poster Presentation

Strategies and prognosis of inflammatory myopathy related cardiomyopathy

Dr Liu Yingxian
Chinese Academy of Medical Sciences and Peking Union Medical College

Abstract

Objective

Although heart failure is an important cause of mortality, rare cases of inflammatory myopathy related cardiomyopathy have been reported. This study aim to investigate different strategies on the prognosis of inflammatory myopathy related cardiomyopathy.

Method

Single center and case control study.

Result

32 patients were involved, including 12 of multiple myositis, 10 of dermatomyositis, 8 of overlap syndrome and 2 of nonspecific myositis. All the patients were prescribed with enough dose of prednisone (1-2 mg/kg/day) at the beginning. 7 cases received intravenous gamma globulin, while 12 cases once underwent high dose steroid pulse therapy. Immunosuppressants were prescribed as follows: 9 with methotrexate, 7 with cyclophosphamide, 2 with cyclosporin A, while 12 cases with combined immunosuppressive medications. For the heart failure management, 24 cases with β receptor blockers, 16 cases with angiotensin converting enzyme inhibitor/angiotensin receptor blockers and 17 cases with spironolactone. 3 patients were lost, the remaining 29 patients with a mean follow-up of 2.5 years (1 month ~ 13 years), deaths were recorded in 14 cases (48.3%), including 9 events of cardiac death. 10 cases suffered from re-admissions because of cardiovascular events and 6 cases suffered from infection complications. Intravenous gamma globulin therapy reduced percentages of cardiac deaths (0/7 vs 9/22, p = 0.013) and all-cause mortality (1/7 vs 13/22, p = 0.031), while steroid pulse therapy significantly reduced readmission percentage of cardiac events (0/12 vs 10/17, p = 0.001) and mortality of cardiac events (1/12 vs 8/17, p = 0.019), when compared with therapy without these medications, meanwhile, the risk of infection readmission did not increase (4/12 vs 2/17). Cardiovascular readmission percentage and all-cause mortality were significantly decreased in combined immune suppressors treatment subgroup (methotrexate plus cyclophosphamide/cyclosporine A) comparing with immune suppressants monotherapy subgroup (p < 0.05). In medications of improving cardiac remodeling, only β receptor blockers prolonged the survival time significantly (Treatment within or without β receptor blockers: 5.6±0.9 years vs. 2.9 ±0.7 years) (Mean + SE, p = 0.030). There was significant difference in survival rate between patients with or without β receptor blockers (Log-Rank chi-square = 4.589, p = 0.032, Kaplan-Meier analysis).

Conclusion

Patients with inflammatory myopathy related cardiomyopathy manifest short survival time and high cardiac mortality. Intravenous gamma globulin, high dose steroid pulse therapy, combination of methotrexate plus other immune suppressants, and long-term administration of β receptor blockers could reduce percentage of cardiac events significantly. We recommend a management that emphasize both primary disease controlling and cardiac remodeling improvement.