Key result
Fulminant systemic sclerosis myocarditis proves fatal despite aggressive immunosuppression and mechanical circulatory support.
Why the study?
Cardiac involvement in systemic sclerosis is under-recognized and can present as fulminant myocarditis with cardiogenic shock and poor outcomes.
Case Report (n=1)
No
Fulminant SSc-myocarditis carries a grave prognosis and requires early recognition and coordinated multidisciplinary care.
BACKGROUND: Cardiac involvement in systemic sclerosis (SSc) is under-recognized and may present as fulminant myocarditis with cardiogenic shock and poor outcomes. CASE SUMMARY: A 49-year-old woman with diffuse SSc presented with progressive dyspnea and hypotension. Laboratory testing showed markedly elevated cardiac biomarkers, and echocardiography demonstrated severe biventricular systolic dysfunction. Electrocardiography revealed sinus tachycardia with right bundle branch block. Cardiac magnetic resonance showed diffuse biventricular myocardial inflammation, confirmed by endomyocardial biopsy demonstrating lymphocytic infiltration. DISCUSSION: Despite inotropes, diuretic agents, and dual mechanical circulatory support with venoarterial extracorporeal membrane oxygenation and an Impella CP device, myocardial recovery did not occur. High-dose immunosuppression was delayed, and heart transplantation was deferred because of cavitary lung lesions. The patient developed progressive multiorgan failure and died in the sixth week of hospitalization. TAKE-HOME MESSAGE: Fulminant SSc-myocarditis carries a grave prognosis and requires early recognition and co-ordinated multidisciplinary care.
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Mohammad et al. (2026) conducted a case report in Systemic sclerosis-associated fulminant myocarditis with cardiogenic shock (n=1). Immunosuppressive therapy and mechanical circulatory support was evaluated on Clinical course and survival. Fulminant systemic sclerosis-associated myocarditis resulted in rapidly progressive biventricular failure, refractory cardiogenic shock, and death despite aggressive immunosuppression and mechanical circulatory support.
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