Early decline in LV systolic function in adult onset of dermatomyositis

Introduction: Dermatomyositis (DM) is an auto-immune myopathy that displays a wide range of clini-cal manifestations, including cardiac involvement. The long-term survival of DM patients is shorter compared to the general population. Increased cardiac mortality was associated with congestive heart failure. Also, coro-nary artery disease is more frequent in rheumatologic diseases than in the general population. Accelerated atherosclerosis in these populations is considered to be related to the associated inflammatory condition, increased prevalence of cardiovascular risk factors and side effects of corticosteroid therapy. We present the case of a recently diagnosed dermatomyositis patient with fast evolution towards severe heart failure.

Methods: We present the case of a 65-year-old woman, recently diagnosed with DM, without other cardiovas-cular risk factors, presenting for chest pain accompa-nied by ECG alterations and regional ecocardiographic wall motion abnormalities. Diagnostic coronarography reveals trivascular involvement requiring angioplas-ty and stenting, with good result. Subsequently, after one year, the patient returns accusing dry coughs and dyspnoea. Echocardiography reveals severely impaired systolic function with new wall motion abnormalities, aggravated mitral regurgitation, which is why the co-ronarography is repeated but with patent stents. The series of cardiac events continues, in a few days she returned for new onset atrial fibrillation, subsequently complicated by embolic stroke under the anticoagulant treatment.

Results: DM is an inflammatory myopathy that freq-uently affects skeletal muscles with extremely rare cardiac involvement. However, it should be clinically recognized for prognostic significance. The main me-chanisms responsible for cardiac involvement in DM appear to be atherosclerosis, inflammation and / or myocardial fibrosis. The presence of severe systolic car-diac dysfunction in pacients with adult onset of DM is well documented in various clinical cases. In the present case, coronary involvement in a patient wi-thout other cardiovascular risk factors supports DM involvement in early atherosclerosis. If atherosclerosis forms the basis of coronary involvement in DM, other mechanisms such as myocardial inflammation and / fi-brosis, systemic inflammation or vasculitis play equally important roles. Early Decline in Left Ventricular Ejec-tion Fraction without recurrent ischemic heart disease, can also be explained by myocardial damage in the DM context. By excluding other causes of impaired systolic function, the only plausible etiology remains the con-text of autoimmune disease.

Conclusions: Even if significant heart involvement is uncommon, cardiac disease is one of the major ca-uses of DM death. Recent studies show an increased prevalence of traditional cardiovascular risk factors in DM, which requires particular attention. Interactions between proinflammatory cytokines and traditional risk factors may contribute to the pathogenesis of car-diac dysfunction. Heart failure may also be related to myocarditis and / or myocardial fibrosis, leading to ar-rhythmias and congestive heart failure, demonstrated in both adult and younger patients.

ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
The Romanian Journal of Cardiology is indexed by:
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CODE: 379
CME Credits: 10 (Romanian College of Physicians)