Abstract Rationale PH is associated with decreased functional capacity, increased risk of hospitalizations, and death in patients with ILD. These patients likely have cardiovascular comorbidities contributing to an increased PH risk that may lead to worse prognosis. This study assessed long-term, all-cause and heart failure (HF)-related hospitalizations, mortality, and comorbidities in patients with connective tissue disease (CTD)-related ILD and non-CTD-related ILD, with/without PH. Methods This retrospective cohort study used the NorstellaLinQ closed claims database to identify claims for ILD with/without CTD, collected between 1-Jan-2018 and 31-Jan-2020 in the US. Patients aged ≥18 years with ≥12 months continuous enrollment before and ≥5 years after index (date of first ILD claim) were included. PH diagnosis was ascertained with claims for PH. A claim for an echocardiogram was also required. All-cause mortality was assessed without requirement of ≥ 5 years continuous enrollment after index. Results In total, 10,898 patients with CTD-ILD and 39,026 patients with non-CTD-ILD were identified. Of these, 2162 (19.8%) in the CTD-ILD cohort and 6184 (15.8%) in the non-CTD-ILD cohort had PH. Patients with PH versus without PH were older (mean SD 57.1 13.7 vs 51.7 15.1 years) and more likely to have comorbidities. A greater proportion of patients with CTD-ILD and PH versus without PH had all-cause and HF-related hospitalizations at baseline, 1-, 3-, and 5-year follow-ups (P0.0001 all), with percentage-point differences between groups from 6% to 23% (Figure). A similar trend was observed for the non-CTD-ILD cohort, with percentage-point differences between groups from 9% to 36% (Figure). PH was associated with greater likelihood of all-cause and HF-related hospitalizations following multivariate analyses adjusting for baseline comorbidities in both cohorts. A greater proportion of patients with PH versus without PH died (CTD-ILD: 19.6% vs 9.9%; non-CTD-ILD: 27.8% vs 16.6%, respectively). More patients with PH versus without PH in both cohorts reported comorbidities, with the highest percentage-point difference between groups for congestive heart failure at the 5-year follow-up (CTD-ILD cohort, 26%; non-CTD-ILD cohort, 38%). Higher mortality rates were observed in comorbidity subgroups with PH for both cohorts. Conclusions In this retrospective analysis, patients with CTD-ILD and non-CTD-ILD with PH had significantly higher all-cause and HF-related hospitalizations and mortality rates than patients without PH. Patients with PH also had more comorbidities associated with worse prognosis and higher mortality. These findings indicate that PH appears to add an additional, significant clinical burden in patients with ILD. This abstract is funded by: This study was funded by Insmed Incorporated (Bridgewater, NJ, USA). Analyses were conducted by Panalgo (Boston, MA, USA) and funded by Insmed Incorporated. All authors contributed to and approved the abstract; writing and editorial assistance were provided by Emanuela Marcantoni, PhD, of The Lockwood Group (Stamford, CT, USA), funded by Insmed Incorporated.
Safdar et al. (Fri,) studied this question.