Abstract Introduction Pulmonary hypertension (PH) is a common complication of idiopathic pulmonary fibrosis (IPF) and is associated with increased morbidity and mortality. No vasodilator therapy was approved for IPF-PH until April 2021, when inhaled treprostinil gained FDA approval based on randomized trial evidence demonstrating clinical benefit. We aimed to assess the uptake of inhaled Treprostinil among patients with IPF-PH following its approval. Methods We performed a retrospective cohort study using TriNetx, which contains de-identified electronic health records and claims-based data from over 130 million patients at 104 health care organizations. We identified adults (⩾ 50 years) with a new diagnosis of IPF-PH from 2016-2024 following as outpatients in the dataset with the following criteria 1) at least two ambulatory visits for IPF (ICD-10 J84.112), 2) at least one ambulatory visit for a non-IPF diagnosis prior to first IPF encounter, and 3) at least two ambulatory visits for PH (ICD-10 I27.x) after the first IPF visit. The ICD-10 code algorithms used have been previously validated for use in administrative data. Patients were excluded if they had diagnosis codes for other ILDs or rheumatologic diseases. We calculated overall rate of time to PH diagnosis, overall rate of inhaled treprostinil use, time to treprostinil use relative to IPF-PH diagnosis period, and quarterly rates of treprostinil uptake relative to quarterly IPF-PH incidence. Results There were 1,115 patients with IPF-PH (median age at IPF diagnosis: 73 IQR 67-78; male: 710 64%; White: 841 75%; non-Hispanic: 825 74%) at 52 healthcare organizations included in our study. Patients were diagnosed with IPF-PH a median of 167 days (IQR 49-563 days) after their initial IPF diagnosis. There were 55 patients (4.9%) who received Treprostinil overall. Of 526 patients diagnosed with PH before April 2021, 12 (2.3%) initiated Treprostinil, compared with 43 of 589 patients (7.3%) diagnosed after FDA approval. The median time from IPF-PH diagnosis to Treprostinil initiation was 805 days (IQR 151-1,172) for those diagnosed before April 2021 versus 42 days (IQR 11-144) for those diagnosed afterward. After April 2021, the quarterly rate of Treprostinil uptake relative to incident IPF-PH diagnoses increased significantly from 4.4% to 20.0% (p for trend=0.007) (Figure). Conclusion Inhaled treprostinil use among IPF-PH patients is low overall, but has increased significantly since FDA approval of the therapy in April 2021. Future studies should understand how to improve uptake of treprostinil among patients with IPF-PH and whether use is sustained overtime. This abstract is funded by: None
Patel et al. (Fri,) studied this question.