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Updated: Jun 24, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Exercise pulmonary hypertension in patients with systemic sclerosis based on updated guidelines
Yu Kanazawa1, Ayumi Goda2, Takato Mohri1
1Department of Cardiovascular Medicine, Kyorin University Hospital, 6-20-2 Shinkawa, Mitaka, Tokyo, 181-8611, Japan.
Exercise pulmonary hypertension (ex-PH) in systemic sclerosis (SSc) patients may be an intermediate stage between no pulmonary hypertension (PH) and overt PH. This finding helps understand ex-PH in SSc according to new guidelines.
Area of Science:
- Cardiology
- Pulmonology
- Rheumatology
Background:
- Recent European guidelines introduced exercise pulmonary hypertension (ex-PH).
- Clinical characteristics of ex-PH in systemic sclerosis (SSc) are not well understood.
- SSc is a connective tissue disease that can affect the lungs.
Purpose of the Study:
- To investigate the clinical characteristics of ex-PH in patients with SSc.
- To compare exercise tolerance and echocardiography results among patients with overt PH, ex-PH, and no PH.
- To determine if ex-PH represents an intermediate condition in SSc.
Main Methods:
- Retrospective study of 77 SSc patients undergoing symptom-limited exercise testing with right heart catheterization.
- Exclusion of 19 patients with postcapillary PH.
- Division of 58 patients into overt-PH, ex-PH, and non-PH groups based on hemodynamic criteria.
Main Results:
- Peak oxygen consumption was highest in the non-PH group, intermediate in the ex-PH group, and lowest in the overt-PH group (p=0.043).
- The minute ventilation/peak carbon dioxide production slope was intermediate in the ex-PH group (p=0.003).
- The tricuspid annular plane systolic excursion/systolic PAP ratio decreased progressively from non-PH to ex-PH to overt-PH (p=0.018).
Conclusions:
- Exercise pulmonary hypertension (ex-PH) in SSc patients may represent an intermediate condition between no PH and overt PH.
- Findings support the new guidelines' classification of ex-PH.
- Further research is warranted to explore the clinical implications of ex-PH in SSc.
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