Abstract
BackgroundPirfenidone is an antifibrotic agent approved for the treatment of idiopathic pulmonary fibrosis (IPF). The drug is available for Polish patients with IPF since 2017. The PolExPIR study aimed to describe the real-world data (RWD) on the Polish experience of pirfenidone therapy in IPF with respect to safety and efficacy profiles.MethodsThis was a multicentre, retrospective, observational study collecting clinical data of patients with IPF receiving pirfenidone from January 2017 to September 2019 across 10 specialized pulmonary centres in Poland. Data collection included baseline characteristics, pulmonary function tests (PFTs) results and six-minute walk test (6MWT). Longitudinal data on PFTs, 6MWT, adverse drug reactions (ADRs), treatment persistence, and survival were also collected up to 24 months post-inclusion.ResultsA total of 307 patients receiving pirfenidone were identified for analysis. The mean age was 68.83 (8.13) years and 77% were males. The median time from the first symptoms to IPF diagnosis was 15.5 (9.75–30) months and from diagnosis to start of pirfenidone treatment was 6 (2–23) months. Patients were followed on treatment for a median of 17 (12–22.75) months. Seventy-four patients (24.1%) required dose adjustments and 35 (11.4%) were chronically treated with different than the full recommended dose. A total of 141 patients (45.92%) discontinued therapy due to different reasons including ADRs (16.61%), death (8.79%), disease progression (6.51%), patient’s own request (5.54%), neoplastic disease (3.91%) and lung transplantation (0.33%). Over up to 24 months of follow-up, the pulmonary function remained largely stable. The median annual decline in forced vital capacity (FVC) during the first year of pirfenidone therapy was −20 ml (−200–100) and during the second year was −120 ml (−340–30). Over a study period, 33 patients (10.75%) died.ConclusionsThe PolExPIR study is a source of longitudinal RWD on pirfenidone therapy in the Polish cohort of patients with IPF supporting its long-term acceptable safety and efficacy profiles and reinforce findings from the previous randomised clinical trials and observational studies.
Highlights
Pirfenidone is an antifibrotic agent approved for the treatment of idiopathic pulmonary fibrosis (IPF)
In almost 85% of subjects IPF was diagnosed based on the presence of a definite usual interstitial pneumonia (UIP) pattern on the high-resolution computed tomography (HRCT) and the remaining of patients required a combination of HRCT and lung biopsy for the definite IPF diagnosis, see Table 1
Transbronchial lung biopsy (TBLB) was used in the diagnostic work-up in as many as 9 patients but the results were diagnostic for UIP only in 2 cases
Summary
Pirfenidone is an antifibrotic agent approved for the treatment of idiopathic pulmonary fibrosis (IPF). Pirfenidone and nintedanib, have been shown to slow the disease progression limiting the decline of lung function in patients with IPF [4,5,6]. Both antifibrotics are recognized as an actual standard of pharmacological treatment of the disease [7]. Recent changes in the reimbursement policy for antifibrotics in Poland have resulted in much wider availability of pirfenidone (since January 2017) and nintedanib (since March 2018) for the treatment of Polish patients with IPF
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
