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Cardiac Manifestations of Systemic Sclerosis (SSc)

This infographic explores the cardiac involvement in systemic sclerosis (SSc), a chronic autoimmune disease characterized by skin thickening, vasculopathy, internal organ fibrosis, and autoantibodies. Cardiac involvement is often subtle but can have significant morbidity and mortality.

Key Points

• Often Underdiagnosed: Up to 70% of patients may have subclinical cardiac involvement, with 10-30% developing symptomatic disease.
• Screening and Monitoring: Recommended tests include ECG, TTE, BNP, troponin, uric acid, and pulmonary function tests (PFTs) at diagnosis and annually.

Cardiac Pathophysiology in SSc

• Microvascular Disease:
• Raynaud’s phenomenon and microvascular ischemia are common.
• Angina may occur, and calcium channel blockers are preferred over beta-blockers, which can worsen Raynaud’s symptoms.
• Myocardial Involvement:
• Can lead to diastolic dysfunction, progressing to LV and RV failure.
• Myocarditis (acute or chronic) and PAH/RV dysfunction affect 5-9% of patients.
• Conduction System Abnormalities:
• Includes autonomic dysfunction, heart block, and arrhythmias (e.g., SVT, bradycardia).
• Holter monitoring or cardiac event monitors may be needed for detailed arrhythmia evaluation.
• Pericardial Involvement:
• Pericardial effusion is the most frequent pericardial manifestation, but pericarditis, tamponade, and constrictive pericarditis can also occur.

Treatment Strategies

• Low-dose prednisone with cyclophosphamide is often used for myocarditis and severe cardiac involvement.
• Management of PAH includes vasodilator therapies. Regular monitoring of pulmonary pressures and cardiac function is essential.

Produced by Ronaldo Correa Fabiano

Edited by Dr. Gurleen Kaur
Reviewed by Dr. Amit Goyal, Dr. Mitesh Mukherjee, Dr. Jeffrey Garshick, and Dr. Brian Weber

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