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Updated: Jun 26, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Overlap of coronary disease and pulmonary arterial hypertension in systemic sclerosis
A Komócsi1, T Pintér, R Faludi
1University of Pécs, Heart Institute, H-7624 Pécs, Ifjúság u. 13, Hungary. andras.komocsi@aok.pte.hu
Insights
Systemic sclerosis (SSc) patients often have overlapping cardiac conditions like pulmonary arterial hypertension (PAH) and coronary artery disease (CAD). Invasive testing is crucial for accurate diagnosis and treatment of these complex cardiac manifestations in SSc.
Area of Science:
- Cardiology
- Rheumatology
- Pulmonology
Background:
- Pulmonary arterial hypertension (PAH) is a frequent complication of systemic sclerosis (SSc).
- Coronary artery disease (CAD) symptoms can overlap with PAH, necessitating cardiac catheterization for definitive diagnosis.
- Understanding the overlap between CAD and PAH in SSc patients is critical for appropriate management.
Purpose of the Study:
- To investigate the extent of overlap between coronary artery disease (CAD) and pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc).
Main Methods:
- Non-invasive investigations identified 30 SSc patients with suspected PAH (n=20) or CAD (n=10).
- Right heart catheterization and coronary angiography were performed on these 30 patients.
- Coronary flow reserve (CFR) was assessed using thermodilution.
Main Results:
- PAH was diagnosed in 12/20 suspected PAH patients and 2/10 suspected CAD patients.
- Coronary artery stenosis was found in 9/20 suspected PAH patients and 6/10 suspected CAD patients.
- Severely reduced CFR was observed in 7/20 suspected PAH patients and 3/10 suspected CAD patients.
Conclusions:
- A significant overlap exists between PAH, CAD, and reduced CFR in symptomatic SSc patients.
- Current non-invasive tests lack sufficient sensitivity and specificity to differentiate these cardiac conditions in SSc.
- Invasive approaches like coronary angiography are necessary for accurate characterization and treatment of cardiac involvement in SSc.
Objectives:
Pulmonary arterial hypertension (PAH) is a common complication of systemic sclerosis (SSc). Symptoms of coronary artery disease (CAD) and PAH are closely related and cardiac catheterisation is needed to confirm their diagnosis. The aim of the present work was to investigate of the extent of overlap between CAD and PAH in patients with SSc.
Methods:
Based on non-invasive investigations, 20 patients out of 120 were suspected to have PAH ("suspected PAH" group). Another 10 patients showed signs of coronary disease ("suspected CAD" Group). In these 30 patients, right heart catheterisation and coronary angiography were performed, and the coronary flow reserve (CFR) was assessed by thermodilution technique.
Results:
In the "suspected PAH" and the "suspected CAD" groups, PAH was found in 12/20 and 2/10 cases, and coronary artery stenosis in 9/20 and 6/10 cases, respectively. Severely reduced CFR was revealed in 7/20 and 3/10 cases, respectively.
Conclusions:
PAH, CAD and reduced CFR all show a considerable overlap in symptomatic patients with SSc. The current non-invasive investigations are neither sensitive nor specific enough to make an appropriate distinction between these different disease manifestations. A more invasive approach, such as coronary angiography at the initial catheterisation, is required to properly characterise and treat the different forms of cardiac involvement in SSc.
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