Inhaled Iloprost Shows No Significant Improvement in ILD-PH Risk, Walk Distance, or Hemodynamics in Real-World Cohort

Patients with Pulmonary Hypertension associated with Interstitial Lung Disease (ILD-PH) face high morbidity and mortality, with limited effective therapeutic options. While inhaled treprostinil has emerged as a treatment, real-world data for other therapies, like inhaled iloprost, are scarce. Understanding longitudinal outcomes and risk stratification in routine clinical practice is crucial to address the significant unmet need for improving patient prognosis and quality of life in this complex and progressive condition.

Researchers conducted a single-center retrospective cohort study of 72 patients with ILD-PH who initiated inhaled iloprost between 2020 and 2025. An exploratory comparator group of 15 patients (of whom 14 were included in survival analyses) who did not receive iloprost was also identified. Clinical, functional, and hemodynamic data were collected at baseline and after a median follow-up of 5.3 months (IQR 4.0-9.0). Risk was assessed using COMPERA 2.0, and survival was estimated via Kaplan-Meier analysis.

At a median of 5.3 months post-iloprost initiation, the cohort of 72 patients (71% female; median age 57.0 years) showed no significant changes in key metrics. This included 6-minute walk distance, WHO functional class, BNP levels, mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance (PVR), pulmonary artery wedge pressure (PAWP), or cardiac index. Right atrial pressure increased and mixed venous oxygen saturation decreased modestly during follow-up (both p < 0.05), though clinical significance remains uncertain. Risk stratification remained largely unchanged across COMPERA 2.0 categories. Over a median total follow-up of 19.2 months (IQR 9.2-33.3), 23 deaths (32%) were confirmed among 71 patients with available survival data, and at least one hospitalization occurred in 50% of patients.

Iloprost was discontinued in 26% of patients, most frequently due to intolerance, highlighting significant real-world challenges.