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[Cardiopathy in systemic sclerosis]
1Cartedra di Reumatologia, Seconda Università, Napoli.
Insights
Systemic sclerosis (SSc) frequently affects the heart. Advanced diagnostics reveal preclinical cardiac disease in many SSc patients, emphasizing the need for regular cardiac screening and tailored treatments.
Area of Science:
- Cardiology
- Rheumatology
- Systemic Sclerosis Research
Context:
- Cardiac involvement is common in systemic sclerosis (SSc), encompassing primary scleroderma heart disease and secondary conditions.
- Distinguishing primary SSc heart disease from secondary causes (pulmonary, renal) is crucial for understanding pathophysiology.
- Autopsy findings reveal a higher prevalence of cardiac involvement than clinical detection, highlighting the need for advanced diagnostics.
Purpose:
- To differentiate pathophysiologic mechanisms of cardiac involvement in SSc.
- To explore the diagnostic gap between clinical detection and autopsy findings in SSc patients.
- To investigate the prognostic significance of cardiac involvement and potential therapeutic interventions.
Summary:
- Advanced techniques like Holter ECG and echocardiography detect preclinical SSc heart disease in a significant percentage of patients.
- Asymptomatic patients may exhibit pericardial effusions, myocardial defects (fixed or reversible), or impaired cardiac reserve.
- Clinically evident SSc heart disease and ventricular arrhythmias are associated with poor prognosis, necessitating regular cardiological evaluation.
- Diastolic dysfunction in SSc explains the limited efficacy of inotropic agents, while captopril shows promise in improving cardiac function by addressing vascular disease and fibrosis.
Impact:
- Highlights the importance of comprehensive cardiological work-ups for systemic sclerosis patients.
- Identifies preclinical SSc heart disease, enabling earlier intervention and improved patient outcomes.
- Suggests captopril as a potential therapeutic agent for improving cardiac function in SSc by targeting underlying mechanisms.
Abstract:
Cardiac involvement is quite frequent in systemic sclerosis (SSc). From a pathophysiologic point of view, one must differentiate a primary scleroderma heart disease due to pericardial and/or myocardial and/or small coronary intramyocardial vessel involvement from heart disease secondary to either pulmonary interstitial or vascular involvement (pulmonal cor) or to kidney disease (hypertensive myocardial disease). A significant difference emerges when the prevalence of clinically and standard ECG detected cardiac involvement in SSc patients is compared with that registered at autopsy. In the last years, however, Holter ECG, echocardiography, perfusional scintigraphy and ventriculography have reduced such gap, a preclinical scleroderma heart disease being detected by such techniques in quite a high percentage of SSc patients. Asymptomatic SSc patients may be found to present small pericardial effusions and/or either fixed (fibrosis) or reversible (vascular disease) or both types thallium defects or a defective cardiac functional reserve. Both clinically evident scleroderma heart disease and ventricular arrhythmias have a poor prognostic significance. Therefore, a complete cardiological work-up must be periodically carried out in SSc patients. Scleroderma heart disease has long been considered a condition difficult to treat. The detection of diastolic abnormalities and of diastolic failure in SSc patients make us able to understand the therapeutic failure of inotropic agents. Recently, captopril has been shown to improve cardiac function in SSc. It might act either on vascular disease or on fibrosis by affecting the remodelling process of the myocardial wall.