Evaluation of Pulmonary Fibrosis Outcomes by Race and Ethnicity in US Adults

Abstract

ImportancePulmonary fibrosis (PF) is characterized by progressive scarring of lung tissue and poor survival. Racial and ethnic minority populations face the greatest risk of morbidity and mortality from disparities impacting respiratory health, but the pattern of age at clinically relevant outcomes across diverse racial and ethnic populations with PF is unknown.ObjectiveTo compare the age at PF-related outcomes and the heterogeneity in survival patterns among Hispanic, non-Hispanic Black, and non-Hispanic White participants.Design, Setting, and ParticipantsThis cohort study included adult patients with a PF diagnosis and used data from prospective clinical registries: the Pulmonary Fibrosis Foundation Registry (PFFR) for the primary cohort and registries from 4 geographically distinct tertiary hospitals in the US for the external multicenter validation (EMV) cohort. Patients were followed between January 2003 and April 2021.ExposuresRace and ethnicity comparisons between Black, Hispanic, and White participants with PF.Main Outcomes and MeasuresAge and sex distribution of participants were measured at the time of study enrollment. All-cause mortality and age at PF diagnosis, hospitalization, lung transplant, and death were assessed in participants over 14 389 person-years. Differences between racial and ethnic groups were compared using Wilcoxon rank sum tests, Bartlett 1-way analysis of variance, and χ2 tests, and crude mortality rates and rate ratios were assessed across racial and ethnic categories using Cox proportional hazards regression models.ResultsIn total, 4792 participants with PF were assessed (mean [SD] age, 66.1 [11.2] years; 2779 [58.0%] male; 488 [10.2%] Black, 319 [6.7%] Hispanic, and 3985 [83.2%] White); 1904 were in the PFFR and 2888 in the EMV cohort. Black patients with PF were consistently younger than White patients (mean [SD] age at baseline, 57.9 [12.0] vs 68.6 [9.6] years; P < .001). Hispanic and White patients were predominantly male (Hispanic: PFFR, 73 of 124 [58.9%] and EMV, 109 of 195 [55.9%]; and White: PFFR, 1090 of 1675 [65.1%] and EMV, 1373 of 2310 [59.4%]), while Black patients were less likely to be male (PFFR, 32 of 105 [30.5%] and EMV, 102 of 383 [26.6%]). Compared with White patients, Black patients had a lower crude mortality rate ratio (0.57 [95% CI, 0.31-0.97), but for Hispanic patients, the mortality rate ratio was similar to that of White patients (0.89; 95% CI, 0.57-1.35). Mean (SD) hospitalization events per person were highest among Black patients compared with Hispanic and White patients (Black: 3.6 [5.0]; Hispanic, 1.8 [1.4]; and White, 1.7 [1.3]; P < .001). Black patients were consistently younger than Hispanic and White patients at first hospitalization (mean [SD] age: Black, 59.4 [11.7] years; Hispanic, 67.5 [9.8] years; and White, 70.0 [9.3] years; P < .001), lung transplant (Black, 58.6 [8.6] years; Hispanic, 60.5 [6.1] years; and White, 66.9 [6.7] years; P < .001), and death (Black, 68.7 [8.4] years; Hispanic, 72.9 [7.6] years; and White, 73.5 [8.7] years; P < .001). These findings remained consistent in the replication cohort and in sensitivity analyses within prespecified deciles of age groups.Conclusions and RelevanceIn this cohort study of participants with PF, racial and ethnic disparities, especially among Black patients, were found in PF-related outcomes, including earlier onset of death. Further research is essential to identify and mitigate the underlying responsible factors.

Health Care Disparities in Pulmonary Fibrosis—Time to Move the Needle Forward

“…Within this cohort, patients were also stratified by type of PF, addressing the most common types: connective tissue disease–related interstitial lung disease (CTD-ILD), idiopathic PF (IPF), and fibrotic hypersensitivity pneumonitis (fHP). Adegunsoye at al reported that within this cohort, Black patients more commonly had CTD-ILD; Hispanic patients, fHP; and White patients, IPF.…”

“…While Adegunsoye at al did not comment on disparities in therapy, interestingly in both cohorts in their study, very few patients in all groups were taking antifibrotic agents, and a substantial number of Black patients were taking corticosteroids, which in themselves can cause considerable morbidity. This may have been a confounding factor in the noted increased time to hospitalization and possibly the suggested increased mortality as well.…”

“…To date, in PF, most of the genomic studies have been conducted in IPF, which occurs predominantly in White men. The study by Adegunsoye at al 1 was not designed to study genetics, but the results suggest this question should be addressed at the intersection of race and ethnicity, sex, gender, socioeconomic status, and diagnosis to fully understand how these may also affect outcomes.…”

“…Adegunsoye at al performed a retrospective analysis of outcomes in patients with pulmonary fibrosis (PF), stratified by age and by race and ethnicity. Using a large cohort of 1904 patients from the Pulmonary Fibrosis Foundation’s extensive database of patients with PF from across the US, they validated that cohort with their own registry encompassing 4 separate centers (n = 2888).…”

Racial Disparities Among Lung Transplant Survivors—Shadows and Substance

“…This pattern is consistent with that seen in pulmonary fibrosis, the leading indication for lung transplant. 6 These findings highlight the urgent need to further investigate and address the impact of health care disparities on lung transplant outcomes for racial and ethnic minority populations. The work by Lehr and colleagues 2 also suggests that the increased risk of posttransplant death among Black recipients may be associated with their region of residence.…”