Incidence Rate and Prevalence of Idiopathic Pulmonary Fibrosis in the United States 2017–2022

Rationale Idiopathic pulmonary fibrosis (IPF) is an incurable chronic progressive fibrotic lung disease with median survival of 3-5 years from diagnosis. Estimates of incidence rate and prevalence of IPF are outdated and vary widely due to differences in study methodology and diagnostic definitions. Objectives To provide an up-to-date estimate of IPF incidence rates and prevalence and describe the comorbidity burden for people with IPF in the United States (US). Methods This non-interventional retrospective study used claims data from the Optum^® Clinformatics^® Data Mart database from 2017 to 2022. Individuals were required to have at least 365-day continuous enrollment in the database to enter the study. Individuals with IPF were identified based on claims with the International Classification of Diseases, Tenth Revision code for IPF (J84.112) following study entry. IPF definitions varied by requirement for one or two qualifying IPF claims and computed tomography scan of the chest and/or lung biopsy. In this analysis, three primary case definitions and two sensitivity analysis definitions were used to identify individuals with IPF. Results By primary case definitions, the overall crude and age- and sex-adjusted incidence rates (per 100,000 person-years) were 14.5-26.1 and 9.8-18.4, respectively; the crude and adjusted prevalences (per 100,000 persons) were 46.3-88.9 and 34.4-67.1, respectively. Incidence rate and prevalence increased markedly with age and varied with ethnicity and region. The most frequent comorbidities were systemic hypertension, chronic obstructive pulmonary disease, and coronary artery disease. Conclusions This study provides an up-to-date summary of the incidence rate and prevalence of IPF in the US and shows a substantial burden of comorbidities among patients diagnosed with IPF.