Chest
Diffuse Lung Disease: Original ResearchFamily History of Pulmonary Fibrosis Predicts Worse Survival in Patients With Interstitial Lung Disease
Section snippets
Methods
This retrospective investigation was conducted at the University of Texas Southwestern (UTSW) and the University of California at Davis (UCD) and was approved by the institutional review board at each institution (UTSW Identifiers: 082010-127 and 092017-007; UCD Identifier: 875917). Consecutive patients with a diagnosis of ILD consenting to clinical registry enrollment at each institution were screened (UTSW, 2003 through 2019; and UCD, 2016 through 2019). All included patients had longitudinal
Results
Of the 1,262 patients included in the analysis, 534 (42%) carried a diagnosis of IPF ILD and 728 (58%) carried a diagnosis of non-IPF ILD, including 234 (32%) with CTD-associated ILD, 197 (27%) with CHP, and 297 (41%) with uILD (Fig 1). The mean age ranged between 57 and 68 years. IPF patients were predominantly men and CTD-associated ILD patients were predominantly women, whereas CHP and uILD patients showed nearly equal sex ratios. Race for all subtypes predominantly was White, and roughly
Discussion
In this multicenter study, we showed that a self-reported family history of pulmonary fibrosis predicts reduced TFS for patients with IPF and non-IPF ILD. Importantly, although a positive family history is present less frequently in patients with non-IPF forms of ILD, when it is identified, the patients have a risk of TFS similar to that associated with sporadic IPF. Although accurately classifying ILD subtype has important implications for disease management, this finding argues that the
Conclusions
A family history of ILD is more common in patients with IPF, but is present across a wide variety of ILDs and predicts worse survival in patients with IPF and non-IPF forms of ILD. Although widespread clinical genetic testing may identify specific prognostic genetic markers, it has not been implemented widely for ILD patients. Our study argues for the systematic ascertainment of detailed family histories for all patients with ILD to inform prognosis and expected disease behavior.
Acknowledgments
Author contributions: J. M. O. is the guarantor of the content of the manuscript, including the data and analysis. J. M. O. and C. A. N. had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. C. C. C., C. K. G., J. M. O., and C. A. N. were responsible for study design, interpretation of results, and the writing of the manuscript. W. S. B., N. D., and J. V. P. contributed to data collection and interpretation
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Cited by (0)
FUNDING/SUPPORT: C. K. G. is supported by the National Institutes of Health [Grant R01HL093096]. W. S. B. is supported by the National Institutes of Health [Grant T32HL007013]. J. M. O. is supported by the National Heart, Lung, and Blood Institute [Grant K23HL138190]. C. A. N. is supported by the National Heart, Lung, and Blood Institute [Grant K23HL148498] and by the National Center for Advancing Translational Sciences [Grant UL1TR001105].
J. M. Oldham and C. A. Newton contributed equally to this manuscript.