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Updated: Jul 1, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Screening for PAH in patients with systemic sclerosis: focus on Doppler echocardiography
J Sánchez-Román1, C F Opitz, O Kowal-Bielecka
1Collagenosis and Pulmonary Hypertension Unit, University Hospital Virgen delRocío, Seville, Spain. sanchezroman@nacom.es
Insights
Patients with systemic sclerosis (SSc) have a high risk of pulmonary arterial hypertension (PAH). Early screening using Doppler echocardiography is recommended to improve outcomes for SSc-PAH patients.
Area of Science:
- Rheumatology
- Cardiology
- Pulmonology
Background:
- Systemic sclerosis (SSc) is a connective tissue disease (CTD) associated with a significant risk of developing pulmonary arterial hypertension (PAH).
- Patients with co-existing SSc and PAH face a poorer prognosis compared to those with either condition alone.
- Given the high prevalence of PAH in SSc and the availability of treatments, systematic screening is crucial.
Purpose of the Study:
- To review current pulmonary arterial hypertension (PAH) guideline recommendations for screening patients with systemic sclerosis (SSc).
- To focus on studies utilizing Doppler echocardiography for screening in SSc-PAH.
- To summarize risk factors and parameters for identifying high-risk SSc patients.
Main Methods:
- Review of current PAH guidelines.
- Analysis of studies employing Doppler echocardiography for SSc-PAH screening.
- Summarization of characteristics and parameters for high-risk patient identification.
Main Results:
- Systematic screening for PAH in SSc is recommended.
- Doppler echocardiography is a key screening tool, but has limitations.
- Specific patient characteristics and parameters aid in identifying high-risk individuals.
Conclusions:
- Early detection of PAH in SSc patients is vital.
- Doppler echocardiography plays a role in screening, with awareness of its limitations.
- Identifying high-risk SSc patients facilitates timely intervention and management.
Abstract:
It is well established that patients with CTDs such as SSc carry a considerable risk of developing pulmonary arterial hypertension (PAH). Such SSc-PAH patients have an even worse prognosis than patients with only one of these two conditions. In view of the high incidence and prevalence of PAH in SSc, and the available treatment options that improve quality of life, exercise capacity and possibly survival, systematic screening has been recommended. The present article reviews current recommendations from PAH guidelines, focusing on studies that used Doppler echocardiography for screening, and describes limitations associated with the procedure. Furthermore, characteristics and parameters used to identify patients at high risk of developing PAH are summarized.
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