[Heart involvement in systemic sclerosis: analysis of four cases]

Area of Science:

• Cardiology
• Rheumatology
• Immunology

Background:

• Systemic sclerosis (SSc) is a multisystem autoimmune disease with high rates of cardiac involvement (80-100%).
• Cardiac complications, including myocardial fibrosis and vascular microangiopathy, are significant causes of mortality in SSc.
• Pulmonary arterial hypertension can lead to right heart failure in end-stage SSc.

Observation:

• This study analyzed four cases of SSc with severe, fatal cardiovascular complications.
• Cases included limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) with pulmonary arterial hypertension, interstitial lung disease, cardiomyopathy, and severe arrhythmias.
• Despite aggressive therapies, including vasodilators, immunosuppressants, and device implantation, patients experienced disease progression and fatal outcomes.

Findings:

• Lung and heart involvement are primary drivers of poor prognosis in SSc.
• Arrhythmias and right heart failure, often associated with cardiomyopathy or pulmonary hypertension, are key causes of cardiac death in SSc.
• Fatal cardiovascular complications are more prevalent in dcSSc, particularly early in the disease course.

Implications:

• Early identification of cardiovascular manifestations in SSc is a critical priority.
• Understanding the high risk of cardiac death in SSc underscores the need for proactive cardiac monitoring and management.
• This case series highlights the challenges in treating severe cardiovascular complications in SSc and the importance of timely intervention.