Cardiac involvement in systemic sclerosis: differences between clinical subsets and influence on survival

Area of Science:

• Cardiology
• Rheumatology
• Systemic Sclerosis Research

Background:

• Cardiac involvement (CI) is a significant complication of systemic sclerosis (SSc), strongly linked to increased patient mortality.
• Understanding the specific patterns and risks of CI across different SSc subtypes is vital for clinical management.

Purpose of the Study:

• To characterize a cohort of Spanish patients with SSc and CI.
• To compare the clinical, epidemiological, immunological, and therapeutic features of patients with and without CI.
• To analyze differences in CI presentation and survival among limited, diffuse (dcSSc), and sine scleroderma SSc subtypes.

Main Methods:

• Retrospective study of 393 Spanish SSc patients diagnosed between 1976 and 2011.
• Assessment of CI using noninvasive and invasive diagnostic tests.
• Comparative analysis of clinical data, including demographics, immunology, and treatment, between patients with and without CI, and across SSc cutaneous subsets.

Main Results:

• CI was identified in 44% of SSc patients, with mitral regurgitation, conduction abnormalities, and diastolic dysfunction being most common.
• Pericardial involvement and heart failure were more prevalent in dcSSc.
• CI was an independent mortality risk factor (HR 2.1) and accounted for 20% of deaths. Patients with dcSSc developed CI more rapidly, particularly within the first year of SSc onset.

Conclusions:

• CI significantly increases mortality risk in SSc patients.
• The presentation and progression of CI differ across SSc cutaneous subsets, with dcSSc patients experiencing faster onset.
• Routine screening for cardiac involvement at diagnosis and during follow-up is recommended for all SSc patients.