Diagnosis, Death in Pulmonary Fibrosis Seen Earlier in Black Patients

— Disparities in age pervasive, say researchers

Last Updated March 16, 2023
MedicalToday
A chest x-ray of a patient with pulmonary fibrosis

Black patients with pulmonary fibrosis (PF) consistently experienced poor outcomes associated with their disease at earlier ages than other groups, including hospitalization and death, according to U.S. registry data spanning nearly two decades.

In an analysis of patients from the Pulmonary Fibrosis Foundation Registry (PFFR), a PF diagnosis in Black individuals occurred about 10 years earlier, on average, before their white and Hispanic counterparts (P<0.001), reported Ayodeji Adegunsoye, MD, MS, of the University of Chicago Department of Medicine, and colleagues.

"The disparities in age were pervasive and ran through the natural history of PF from diagnosis through disease progression, culminating in early occurrence of hospitalization, lung transplant, and death among racial and ethnic minority populations," the group wrote in .

Mean number of hospitalizations over the study period were highest among Black patients (3.6 per person) compared with Hispanic and white patients (1.8 and 1.7 per person, respectively; P<0.001). And despite a numerically longer mean survival time, Black patients with PF had a younger age at death.

"Any modest gain in life expectancy presumably accrued by Black patients fell short of the age differences, considering that PF diagnosis occurred a decade earlier in Black compared with white patients," wrote Adegunsoye and co-authors. "Earlier onset of disease likely exerts considerable consequences on quality of life, hospitalization frequency, and functional capacity of affected persons."

And consistent with current trends, the researchers said their "study showed lower lung transplant rates and disproportionately higher hospitalization rates among Black and Hispanic patients with PF compared with white patients, further underscoring the impact of healthcare disparities on these racial and ethnic minority populations."

Their study included a total of 4,729 adult patients with PF in the PFFR (n=1,904), as well as registries from four U.S. hospitals used as an external multicenter validation (EMV) cohort (n=2,888). Of these, 83% were white, 10% were Black, and 7% were Hispanic. The average age overall was 66 years, and 58% were men. Overall, patients were followed from 2003 to 2021.

Black patients more commonly had connective tissue disease-related interstitial lung disease (CTD-ILD; 55%) compared with Hispanic (21.8%) and white (13.6%) patients. While white patients were more likely to have idiopathic PF (66%) compared with Hispanic (48%) and Black (16%) patients (P<0.001 for both).

In the PFFR, Black patients with PF were younger at enrollment (Black 57.9 years, Hispanic 65.4 years, white 68.6 years), younger at first hospitalization (59.4, 67.5, and 70 years, respectively), younger at lung transplant (58.6, 60.5, and 66.9 years) and younger at death (Black 68.7 years, Hispanic 72.9 years, white 73.5 years; P<0.001 for all).

In the PFFR, Black patients had a lower crude mortality rate ratio compared with whites (0.57, 95% CI 0.31-0.97), a finding that was not seen in adjusted analysis of the PFFR, but was in the EMV cohort.

In an , Yolanda Mageto, MD, MPH, of the Center for Advanced Heart and Lung Disease at Baylor University Medical Center in Dallas, said the study raises questions about the physiologic processes driving the earlier onset of PF in Black patients: "How much is attributable to the social determinants of health? How much to genetic factors?"

While noting the study's strengths -- number of patients, use of a validation cohort, reliance on centers of excellence for a correct diagnosis -- Mageto also noted the various limitation.

"First, regarding time to diagnosis, patients already may have been diagnosed for some time before arrival at the center, which may result in a lead-time bias," she said. "Second, Black and Hispanic patients were only 16.8% of the cohort and were imbalanced between the subgroups of PF. This may have affected the findings of more frequent hospitalizations and earlier transplant times, given that Black women have been shown to be more prone to develop connective tissue disease and have more frequent hospitalizations. Third, follow-up time was limited to 3 years, limiting assessment of long-term survival."

Other limitations, cited by Adegunsoye and colleagues, included the inability to examine genetic ancestry.

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    Elizabeth Short is a staff writer for . She often covers pulmonology and allergy & immunology.

Disclosures

The study was funded by grants from the National Heart, Lung, and Blood Institute (NHLBI)/NIH. The Pulmonary Fibrosis Foundation (PFF) Registry is supported by Genentech, Boehringer Ingelheim, United Therapeutics, InterMune, and others.

Adegunsoye reported relationships with the NHLBI, NIH, PFF, American College of Chest Physicians, Boehringer Ingelheim, Genentech, and Roche. Coauthors reported various relationships with government, industry, and other non-governmental organizations.

Mageto reported relationships with Boehringer Ingelheim, Genentech, United Therapeutics, and FibroGen.

Primary Source

JAMA Network Open

Adegunsoye A, et al "Evaluation of pulmonary fibrosis outcomes by race and ethnicity in US adults" JAMA Netw Open 2023; DOI: 10.1001/jamanetworkopen.2023.2427.

Secondary Source

JAMA Network Open

Mageto Y N "Health care disparities in pulmonary fibrosis -- time to move the needle forward" JAMA Netw Open 2023; DOI:10.1001/jamanetworkopen.2023.2442.