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Updated: May 20, 2025
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary vascular dysfunction in systemic sclerosis
Mads Jønsson Andersen1, Andreas Husted1, Tor Skibsted Clemmensen1
1Department of Cardiology, Aarhus University Hospital, Aarhus, Denmark.
Insights
Routine screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) may miss cases. Adding the DETECT algorithm and exercise testing improves detection of PAH and exercise-induced pulmonary hypertension in SSc patients.
Area of Science:
- Cardiology
- Rheumatology
- Pulmonology
Background:
- Systemic sclerosis (SSc) is associated with a high risk of pulmonary arterial hypertension (PAH).
- Current screening methods for PAH in SSc may not be fully sensitive.
- Early detection of PAH is crucial for timely intervention and improved outcomes.
Purpose of the Study:
- To evaluate the effectiveness of the DETECT algorithm and invasive hemodynamic exercise testing in identifying missed PAH cases in SSc patients undergoing routine screening.
- To assess the prevalence of exercise-induced pulmonary hypertension and group 2 pulmonary hypertension in this cohort.
Main Methods:
- A single-center study of 73 SSc patients without known PAH.
- Echocardiography and the DETECT algorithm were used for initial screening.
- Invasive hemodynamic assessment at rest and during exercise was performed in a subset of patients.
Main Results:
- The DETECT algorithm identified 3 patients meeting 2022 ESC guidelines for PAH at rest.
- 18/19 patients showed abnormal pulmonary arterial pressure during exercise, with 7/9 having exercise-induced pulmonary hypertension.
- One-quarter of patients had group 2 pulmonary hypertension due to left ventricular dysfunction.
Conclusions:
- Routine PAH screening in SSc may miss cases, highlighting the need for enhanced diagnostic approaches.
- The DETECT algorithm and invasive hemodynamic exercise testing can improve PAH detection in SSc.
- Abnormal pulmonary vascular responses during exercise and group 2 PH are common in SSc patients.
Purpose:
This study aimed to determine if any cases of pulmonary arterial hypertension (PAH) were missed using routine screening of systemic sclerosis (SSc). We assessed the potential benefits of adding the DETECT algorithm and invasive hemodynamic exercise testing into the local screening process in a Danish single-center cohort without known PAH.
Methods:
Seventy-three patients with SSc (aged 60 ± 10 years; 56 (77%) females) underwent echocardiography. Among them, 24/73 had an elevated step 2 score of whom 3 had a high risk of pulmonary hypertension on echocardiographic assessment. Four patients declined right heart catheterization. Twenty patients (aged 62 ± 10 years, 13 (65%) females) underwent invasive hemodynamic assessment at rest. Nineteen patients participated in invasive hemodynamic exercise testing.
Results:
At rest, 3/20 patients met the 2022 ESC guidelines for PAH (mean pulmonary arterial pressure (mPAP) >20 mm Hg, pulmonary arterial wedge pressure (PAWP) ≤15 mm Hg, and pulmonary vascular resistance >2 Wood units). According to the 2015 ESC guidelines, only 1/19 patients had PAH. During exercise, 18/19 experienced an mPAP of >30 mm Hg, with the majority (13/19) showing an abnormal pulmonary pressure-flow relationship >3 mm Hg/liter/min. Interestingly, 7 out of 9 patients with no pulmonary hypertension (PH) at rest had exercise-induced pulmonary hypertension. One-quarter of the patients had pulmonary hypertension associated with left ventricular dysfunction (group 2 PH, WHO classification).
Conclusions:
In a Danish cohort of 73 patients with SSc already undergoing bi-annual PAH screening, applying the DETECT algorithm identified 3 patients meeting the 2022 ESC/ERS guideline criteria for PAH at rest, 1 of whom demonstrated increased PAWP with exercise. However, many patients exhibited an abnormal pulmonary vascular response to exercise and/or signs of group 2 PH.
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