An Abbreviated Assessment Tool for Detection of Pulmonary Hypertension in Patients With Interstitial Lung Disease

Chebly Dagher, David M. O'Sullivan, Harrison W. Farber, Raj Parikh

https://doi.org/10.1002/pul2.70316 
 

Abstract

Pulmonary hypertension (PH) is a major complication of interstitial lung disease (ILD) and is associated with increased morbidity and mortality. Early recognition of PH in ILD is crucial for guiding diagnostic evaluation, initiating treatment, and considering lung transplantation. The previously developed PH-ILD Detection Tool by Parikh et al. incorporated eight variables to stratify patients into low-, intermediate-, and high-risk groups, and was subsequently validated in an independent cohort. While effective, the multi-component score may be difficult to implement in routine practice. The aim of this study was to develop a simplified assessment tool for PH in ILD that relies on easily obtainable clinical data. We retrospectively evaluated 154 ILD patients who underwent right heart catheterization. The abbreviated PH-ILD score assigned one point each for: (1) physical examination findings suggestive of PH, (2) diffusion capacity for carbon monoxide < 40% predicted, and (3) supplemental oxygen use (range 0–3). Diagnostic performance was assessed using sensitivity, specificity, predictive values, and an ROC curve. The mean age was 70.0 ± 12.1 years, and the overall prevalence of PH was 48.1%. PH prevalence increased with higher scores: 14.3% (score 0), 58.1% (score 1), 79.0% (score 2), and 87.2% (score 3). The abbreviated tool demonstrated good discriminatory ability (AUC 0.74; 95% CI 0.65–0.82; p < 0.001). A threshold of ≥ 2 yielded a sensitivity of 90%, specificity of 58%, PPV of 78%, and NPV of 79%, while a score of 3 produced higher specificity (88%) with lower sensitivity (54%). This simplified 3-point PH-ILD assessment tool effectively stratifies PH risk in ILD using readily accessible clinical variables. A threshold of ≥ 2 provides high sensitivity for screening, whereas a score of 3 offers improved specificity. This tool may support early identification of PH in ILD and prompt timely referral for specialized evaluation and advanced therapies.

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