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Updated: May 29, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Heart involvement and pulmonary arterial hypertension in patients with systemic sclerosis]
1Zavod za klinikku imunologiju i reumatologiju, Kispatićeva 12,10000 Zagreb.
Insights
Systemic sclerosis frequently affects the heart, leading to poor prognosis and increased mortality. This review covers diagnosis and treatment for cardiac involvement and pulmonary arterial hypertension in scleroderma patients.
Area of Science:
- Cardiology
- Rheumatology
- Pulmonology
Context:
- Systemic sclerosis (SSc) commonly involves the heart, significantly impacting patient prognosis and survival.
- Cardiac manifestations in SSc include myocardial disease, arrhythmias, conduction disturbances, pericardial issues, and right heart failure secondary to pulmonary arterial hypertension (PAH).
- Pulmonary arterial hypertension (PAH) is a critical complication in SSc, characterized by high morbidity and mortality.
Purpose:
- To present diagnostic methods for evaluating cardiac involvement in SSc.
- To outline current diagnostic approaches for pulmonary arterial hypertension (PAH) in SSc patients.
- To discuss contemporary treatment strategies for cardiac involvement and PAH in systemic sclerosis.
Summary:
- Systemic sclerosis is frequently associated with cardiac complications, including myocardial disease and heart failure, often linked to pulmonary arterial hypertension.
- Early diagnosis of cardiac involvement and PAH in SSc is crucial for improving outcomes.
- Current treatment options for these conditions in SSc are evolving and discussed herein.
Impact:
- Highlights the significant impact of cardiac involvement and PAH on systemic sclerosis prognosis.
- Emphasizes the importance of comprehensive diagnostic evaluation for SSc-related cardiovascular complications.
- Provides an overview of current therapeutic interventions, informing clinical practice and future research directions.
Abstract:
In patients with systemic sclerosis heart involvement is often manifest, associated with poor prognosis and increased mortality. Cardiac involvement may be manifested by myocardial disease, arrhhythmias, conduction system disturbances, pericardial abnormalities and right heart failure, which develops as a complication of pulmonary arterial hypertension and significantly influences the disease progression. Pulmonary arterial hypertension is a severe and progressive disease with significant morbidity and mortality, being more often diagnosed. Diagnostic methods in evaluation of heart involvement as well as pulmonary arterial hypertension are presented. Today's treatment modalities are discussed.
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