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Updated: Jan 8, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Prognostic Value of a Cardiopulmonary Exercise Testing-Derived Summed Score in Idiopathic Pulmonary Fibrosis and
Yu-Lin Tsai1,2,3, Kai-Ming Chang4,5, Chung-Shih Chin4,6,7
1Integrated Care Centre of Interstitial Lung Disease, Taichung Veterans General Hospital, Taichung City, Taiwan.
Insights
A cardiopulmonary exercise testing (CPET) score predicts long-term mortality and lung transplant needs in interstitial lung disease (ILD) patients. This prognostic tool is effective for both idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated ILD (CTD-ILD).
Area of Science:
- Pulmonary Medicine
- Cardiopulmonary Exercise Testing (CPET)
- Interstitial Lung Disease (ILD)
Background:
- Previous research indicated a CPET-derived score predicts 1-year mortality in ILD.
- Long-term prognostic value across diverse ILD etiologies was previously unclear.
- Idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated ILD (CTD-ILD) are key ILD subtypes.
Purpose of the Study:
- To evaluate the long-term predictive performance of CPET-derived parameters.
- To assess outcomes in patients with IPF and CTD-ILD.
- To validate the prognostic utility of a CPET-derived summed score.
Main Methods:
- Prospective cohort study of 210 newly diagnosed ILD patients (2018-2022).
- Cardiopulmonary exercise testing (CPET) performed on all participants.
- Cox regression, ROC curve analysis, and Kaplan-Meier survival analysis used to assess predictive ability and event-free survival.
Main Results:
- A CPET-derived summed score (five variables) independently predicted all-cause mortality or lung transplantation.
- Patients with scores of 2-5 had significantly lower event-free survival (44.2%) compared to scores of 0-1 (88.3%).
- The score demonstrated consistent predictive value in both IPF and CTD-ILD cohorts.
Conclusions:
- The CPET-derived summed score is a valuable prognostic tool for ILD patients.
- The score accurately predicts long-term outcomes, including mortality and lung transplantation, in IPF and CTD-ILD.
- External validation in other ILD subtypes is recommended.
Background And Objective:
Our previous study demonstrated that a summed score derived from six cardiopulmonary exercise testing (CPET) parameters could predict 1-year mortality in patients with interstitial lung disease (ILD). However, its long-term prognostic value across different ILD aetiologies remains unclear. This study aimed to assess the predictive performance of CPET-derived parameters for long-term outcomes in patients with idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated ILD (CTD-ILD).
Methods:
In this prospective cohort study, 210 patients newly diagnosed with ILD between 2018 and 2022 at a tertiary medical centre underwent CPET. A CPET-derived summed score was evaluated for its association with a composite outcome of all-cause mortality or lung transplantation. Cox regression and receiver operating characteristic curve analyses were used to examine predictive ability and identify the optimal cutoff value. Kaplan-Meier survival analysis and log-rank tests compared event-free survival in IPF and CTD-ILD patients.
Results:
A summed score incorporating five CPET-derived variables was an independent predictor of the composite outcome. Patients with scores of 2-5 had markedly lower event-free survival (44.2%) than those with scores of 0-1 (88.3%). The score demonstrated consistent predictive value in both IPF and CTD-ILD.
Conclusion:
The CPET-derived summed score is a useful prognostic tool for predicting all-cause mortality or the need for lung transplantation in newly diagnosed ILD patients. It also retains predictive accuracy for long-term outcomes in both IPF and CTD-ILD. External validation in other ILD subtypes is warranted.
Trial Registration:
ClinicalTrials.gov identifier: NCT06476470.
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