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Myocardial fibrosis in polymyositis
H J Anders1, A Wanders, M Rihl
1Medizinische Poliklinik and 2 Pathologisches Institut, Ludwig-Maximilians University, Munich, Germany.
The Journal of Rheumatology
|August 18, 1999
Summary
Symptomatic cardiac disease is rare in polymyositis patients, even with elevated cardiac enzymes. This case highlights severe myocardial fibrosis and heart failure in a polymyositis patient despite immunosuppressive therapy.
Area of Science:
- Cardiology
- Rheumatology
- Pathology
Background:
- Polymyositis, an inflammatory myopathy, can affect the heart, but symptomatic cardiac disease is infrequent.
- Noninvasive studies often suggest myocardial involvement, yet clinical manifestations remain rare.
Observation:
- A 27-year-old woman with a 6-year history of severe polymyositis and elevated creatine phosphokinase-MB isoenzyme presented with sudden congestive heart failure and bradycardia-tachycardia syndrome.
- Despite long-term, intensive immunosuppressive therapy for her polymyositis, her cardiac condition progressed.
Findings:
- Autopsy revealed extensive myocardial fibrosis in the patient.
- Crucially, no inflammatory cell infiltrates were found in the myocardium, despite active skeletal muscle polymyositis.
Implications:
- This case underscores the potential for severe, non-inflammatory cardiac fibrosis in polymyositis, independent of active muscle inflammation.
- It suggests that immunosuppressive therapy may not prevent cardiac fibrosis in polymyositis, necessitating further research into cardiac manifestations and treatment strategies.