Abstract Rationale IPF is a deadly, progressive chronic disease with significant unmet treatment needs. Reported incidence and prevalence estimates are variable. Although antifibrotics are the standard-of-care, they are associated with high discontinuation rates. This study aimed to determine the incidence and prevalence of IPF, and to evaluate the discontinuation rate of antifibrotic therapies among patients with IPF. Methods A targeted literature review using MEDLINE and Embase identified 1,098 eligible observational studies from 2012-2023, of these, 19 (incidence), 19 (prevalence) and 36 (discontinuation rate of antifibrotic therapies) were included. Studies were from China, France, Germany, Italy, Japan, Spain, the United Kingdom, and the United States. To reflect clinical practice, a narrow IPF case definition was used to estimate incidence and prevalence, which identified patients using IPF diagnostic codes (e.g., International Classification of Diseases ICD-9 516.31/ICD-10 J84.112), excluding those with other interstitial lung diseases (ILDs), and requiring confirmatory procedures (e.g., imaging or biopsy). Additionally, age group (0-44, 45-54, 55-64, 65-74, ≥75 years) was assessed as an effect modifier. Timepoint of discontinuation assessment (0-12 or 12 months) and study setting (registry, single-center, multi-center, administrative database) were assessed as effect modifiers for the discontinuation rate of antifibrotics. A random-effects model was used. Generalized linear mixed models estimated measures using a Poisson distribution (incidence) and a binomial distribution (proportions). Sensitivity analyses excluding outlier and low-quality studies were conducted. Results Overall, the estimated pooled incidence rate of IPF was 5.2 per 100,000 person-years and prevalence was 23.9 per 100,000 persons (Table 1). Incidence and prevalence of IPF increased with age. The overall pooled antifibrotics discontinuation rate was 34.5% over a follow-up period of 9.9-52.1 months. With some exceptions, overall pooled effects remained similar after exclusion of outliers and low-quality studies. Pooled antifibrotic discontinuation rates were higher for studies with discontinuation assessment after 12 months (65.9%, 95% confidence interval CI: 43.0%-83.2%) than for those within 0-12 months (34.3%, 95% CI: 15.6%-59.5%). Discontinuation rates were similar across all study settings. Conclusions The incidence (5.2 per 100,000 person-years) and prevalence (23.9 per 100,00 persons) of IPF align with previous studies. Over one third of patients discontinue current antifibrotics, with a higher discontinuation rate after 12 months; discontinuation may be due to adverse events, tolerability or disease progression. Overall, it was observed that antifibrotic treatments are difficult for patients to maintain long-term, highlighting the need for well-tolerated treatments that stabilize IPF. This abstract is funded by: This study was funded by GSK (study number 224043)
Molyneaux et al. (Fri,) studied this question.
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