AI Article Synopsis
- This study focuses on interstitial lung diseases (ILDs) in Japan, specifically looking at the incidence of progressive pulmonary fibrosis (PPF) in patients with fibrosing ILDs that aren't idiopathic pulmonary fibrosis.
- Data from nearly 35,000 patients revealed that 41.7% progressed to PPF within 24 months, with rates being similar across different ILD subtypes, and the most common treatments included oral corticosteroids and tacrolimus.
- The findings highlight a significant prevalence of PPF progression in chronic fibrosing ILDs, emphasizing the need for future research on how regular monitoring and early treatment could improve patient outcomes.
Article Abstract
Background: Interstitial lung diseases (ILDs) are a heterogeneous group of disorders, a subset of which develop progressive pulmonary fibrosis (PPF). There is little information on the epidemiology and treatment of PPFs in Japan. This retrospective cohort study estimated the incidence probability of progression to PPFs in patients with fibrosing ILDs other than idiopathic pulmonary fibrosis in a real-world Japanese setting. Management procedures and treatment patterns were also quantified.
Methods: Data were extracted from the Medical Data Vision database from 01-Jan-2012 to 28-May-2020, comprising a 6.91-year patient identification period, 1-year pre-index period, and post-index period. The primary outcome was the cumulative incidence probability of progression to PPF up to 24 months. Subgroup analyses were performed by the presence/absence of connective tissue disease-ILD and by pre-specified ILD clinical diagnosis.
Results: Of the 34,960 eligible patients (mean age: 71.1 years, males: 52.5%), 14,580 (41.7%) progressed to PPF. The 24-month incidence probability of progression to PPF was 39.5%. A relatively comparable percentage of patients progressed across all ILD subtypes. Oral corticosteroids and tacrolimus were the most common therapies during the pre- and post-index periods. Treatment rates were very low in the post-index period.
Conclusions: This is the first claims database study to estimate the incidence probability of progression to PPF in Japan. Progression appeared common in patients with chronic fibrosing ILDs, with comparable percentages of patients across all subtypes developing PPF at 2 years. Future studies should assess the impact of regular monitoring and early intervention on treating fibrotic ILDs and preventing progression.
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| http://dx.doi.org/10.1016/j.resinv.2024.05.005 | DOI Listing |
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