Shifting Trends in the Epidemiology and Management of Idiopathic Pulmonary Fibrosis in the Era of Evidence-Based Guidelines: a Nationwide Population Study.

Background: Advances in the understanding of idiopathic pulmonary fibrosis (IPF) and international cooperation have led to the publication and subsequent updates of international practice guidelines. The impact of these guidelines, especially significant updates occurring after 2011, on IPF epidemiology and clinical practices remains relatively unexplored.

Methods: This retrospective nationwide population-based study utilized the Whole-Population Datafiles (WPD) of Taiwan's National Health Insurance Research Database that contained basic demographics, complete claim data, and causes of death for all insured persons. We refined the code-based definition to identify IPF cases from the WPD between 2011 and 2019. Independent validation confirmed the high accuracy of this definition. We analyzed the annual standardized rates of IPF incidence, prevalence, overall and IPF-specific all-cause mortality. Additionally, we examined trends in the prescription of selected medications and the proportions of patients with respiratory failure receiving invasive (IMV) and non-invasive (NIV) mechanical ventilation.

Results: We included 4359 incident cases of IPF. From 2011 to 2019, the annual standardized incidence rates increased from 1.66 (95% confidence interval [CI], 1.36-1.97) to 11.35 (95% CI, 10.65-12.04) per 100,000 standard population, and the annual standardized prevalence rates increased from 1.98 (95% CI, 1.65-2.31) to 27.25 (95% CI, 26.17-28.33) per 100,000 standard population. The standardized IPF-specific all-cause mortality and respiratory failure rates remained stable. Male and older patients received IPF diagnoses more frequently, and experienced higher mortality rates, compared to their female and younger counterparts. Most deaths were attributed to respiratory causes, without significant seasonal variation. Changing trends in the management of IPF mirrored with the evolving guideline recommendations, and showed diminishing roles of immunosuppressants, growing usage of antifibrotics, and NIV usage surpassing IMV.

Conclusions: Our findings reflected the longitudinal impact of the recently evolving guideline recommendations on IPF epidemiology and real-world management.