Background Connective tissue disease associated with interstitial lung disease (CTD-ILD) is a major interstitial lung disease with various outcomes. It is crucial to assess the prognosis of CTD-ILD patients. GAP scoring system has been widely used to predict the prognosis of IPF. CT scan and transthoracic echocardiography (TTE) have been applied in evaluating the survival rate in some lung diseases. In this research, we sought to evaluate whether the addition of CT or TTE parameters to GAP score might improve the predictive value of GAP in CTD-ILD patients. Methods Four hundred ten Chinese CTD-ILD patients were enrolled in our research. We retrospectively reviewed the imaging data, TTE data, GAP score and clinical data of the patients, and their clinical outcomes were also determined. Results During the observational period, 58 patients were followed to death. Cox univariate proportional hazard analysis showed that age, gender, erythrocyte sedimentation rate (ESR), diffusing capacity of the lung for carbon monoxide (DLco), mean pulmonary artery (MPA) diameter, right atrial area (RAA), and GAP score were related to all-cause mortality in CTD-ILD patients. In Cox multivariate proportional hazard analysis, MPA, GAP, age, gender, and ESR were predictors of all-cause mortality in patients with CTD-ILD. Kaplan–Meier analysis showed that 2-year and 5-year survival rates were significantly lower in CTD-ILD patients with MPA ≥ 30.00 mm compared with those with MPA < 30.00 mm. In addition, adding MPA to GAP score significantly improved the predictive value compared to the GAP score alone in patients with CTD-ILD. Conclusion MPA accessed by CT is a useful tool for assessing the risk of death in patients with CTD-ILD. In addition, the combination of MPA and GAP score might better identify the prognosis of CTD-ILD patients than GAP score alone.<Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> Keypoints • Although CTD-ILD is a kind of interstitial lung disease with different backgrounds, pulmonary artery size (PA) appears to be an independent predictor of CTD-ILD patients. • Our results showed that in CTD-ILD patients, combination pulmonary artery size and GAP could provide more accurate information for predicting prognosis in CTD-ILD patients than the GAP score alone. </entry> </row> </tbody> </tgroup> </Table>

Risk assessment in connective tissue disease-associated interstitial lung disease: the incremental prognostic value of the size of pulmonary artery

期刊论文
发表日期：

2026年7月29日

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Fu-rui Zhang,
Yan-ping Su,
Han-qiang Zhan,
Li-li Lou,
Yan Fu,
Lei Wang

摘要

Background

Connective tissue disease associated with interstitial lung disease (CTD-ILD) is a major interstitial lung disease with various outcomes. It is crucial to assess the prognosis of CTD-ILD patients. GAP scoring system has been widely used to predict the prognosis of IPF. CT scan and transthoracic echocardiography (TTE) have been applied in evaluating the survival rate in some lung diseases. In this research, we sought to evaluate whether the addition of CT or TTE parameters to GAP score might improve the predictive value of GAP in CTD-ILD patients.

Methods

Four hundred ten Chinese CTD-ILD patients were enrolled in our research. We retrospectively reviewed the imaging data, TTE data, GAP score and clinical data of the patients, and their clinical outcomes were also determined.

Results

During the observational period, 58 patients were followed to death. Cox univariate proportional hazard analysis showed that age, gender, erythrocyte sedimentation rate (ESR), diffusing capacity of the lung for carbon monoxide (DLco), mean pulmonary artery (MPA) diameter, right atrial area (RAA), and GAP score were related to all-cause mortality in CTD-ILD patients. In Cox multivariate proportional hazard analysis, MPA, GAP, age, gender, and ESR were predictors of all-cause mortality in patients with CTD-ILD. Kaplan–Meier analysis showed that 2-year and 5-year survival rates were significantly lower in CTD-ILD patients with MPA ≥ 30.00 mm compared with those with MPA < 30.00 mm. In addition, adding MPA to GAP score significantly improved the predictive value compared to the GAP score alone in patients with CTD-ILD.

Conclusion

MPA accessed by CT is a useful tool for assessing the risk of death in patients with CTD-ILD. In addition, the combination of MPA and GAP score might better identify the prognosis of CTD-ILD patients than GAP score alone.

Keypoints

• Although CTD-ILD is a kind of interstitial lung disease with different backgrounds, pulmonary artery size (PA) appears to be an independent predictor of CTD-ILD patients.

• Our results showed that in CTD-ILD patients, combination pulmonary artery size and GAP could provide more accurate information for predicting prognosis in CTD-ILD patients than the GAP score alone.