C104-19 Patient Reported Outcomes in Interstitial Lung Disease With and Without Pulmonary Hypertension: Interim Results From the PHINDER Study

Abstract Background Despite the poor prognosis of interstitial lung disease (ILD) complicated by pulmonary hypertension (PH), there are no evidence-based guidelines for PH screening in ILD. PHINDER (NCT05776225) aims to develop a validated PH screening methodology in ILD. This interim analysis compared patient-reported outcomes (PROs) in ILD patients with and without PH. Methods PHINDER is a prospective, multicenter study in which patients with ILD undergo routine non-invasive assessments, including PROs, followed by right heart catheterization (RHC). PROs included: Self-reported Functional Class ranging from I (no limitation) to IV (severe limitation); University of California San Diego Shortness of Breath Questionnaire (SOBQ) which grades breathlessness during various activities, with total scores ranging from 0 (no breathlessness) to 120 (maximum breathlessness); and King’s Brief Interstitial Lung Disease (KBILD) questionnaire which includes three domains (Psychological, Breathlessness and activities, and Chest symptoms) and total scores ranging from 0-100 where higher scores indicate better health-related quality of life. Descripted statistics were used to compare results from each questionnaire between patients with pre-capillary PH (mPAP20 mmHg, PVR2 WU, PAWP≤15 mmHg) and those without (mPAP≤20, PVR≤2, PAWP≤15). Results This interim analysis included 134 patients with completed PROs, 105 (78%) with pre-capillary PH and 29 (22%) with normal hemodynamics (Table 1). Patients with ILD and PH reported worse functional class (60% had noticeable or severe limitation vs 45% in those without PH) and numerically worse breathlessness during activity (mean SOBQ total score = 57 vs 42 in those without PH). Health-related quality of life was also numerically worse in patients with ILD and PH (mean KBILD total score = 50 vs 57 in those without PH), which was true across all domains (mean Psychological score = 51 vs 58; Breathlessness Chest Symptoms = 63 vs 67). Conclusions Based on this descriptive analysis, patients with ILD complicated by PH may have more severe physical limitations, worse breathlessness during activity, and decreased health-related quality of life across all domains. These results emphasize the need for timely detection and management of PH in patients with ILD. The final dataset from PHINDER will be used to develop a practical, validated tool to aid in the screening of PH in ILD. This abstract is funded by: United Therapeutics