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Abstract
Background Pirfenidone and nintedanib are the only disease-modifying treatments available for idiopathic pulmonary fibrosis (IPF). Our aim was to test their effectiveness and safety in clinical practice.
Methods This is a single-centre retrospective observational study undertaken at a specialised interstitial lung disease centre in England. Data including progression-free survival (PFS), mortality and drug tolerability were compared between patients with IPF on antifibrotic therapies and an untreated control group who had a forced vital capacity percentage (FVC %) predicted within the licensed antifibrotic treatment range.
Results 104 patients received antifibrotic therapies and 64 control patients were identified. PFS at 6 months was significantly greater in the antifibrotic group (75.0%) compared with the control group (56.3%) (p=0.012). PFS was not significant at 12 or 18 months when comparing the antifibrotic group with the control group. The 12-month post-treatment mean decline in FVC % predicted (−4.6±6.2%) was significantly less than the 12-month pretreatment decline (−10.4±11.8%) (p=0.039). The 12-month mortality rate was not significantly different between the antifibrotic group (25.3%) and the control group (35.5%) (p=0.132). Baseline Body Mass Index of≤25, baseline diffusion capacity for carbon monoxide percentage predicted of ≤35 and antifibrotic discontinuation within 3 months were independent predictors of 12-month mortality. Antifibrotic discontinuation was significantly higher by 3 and 6 months for patients on pirfenidone than those on nintedanib (p=0.006 and p=0.044, respectively). Discontinuation at 12 months was not significantly different (p=0.381).
Conclusions This real-world study revealed that antifibrotics are having promising effects on PFS, lung function and mortality. These findings may favour commencement of nintedanib as first-line antifibrotic therapy, given the lower rates of early treatment discontinuation, although further studies are required to investigate this.
- interstitial fibrosis
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Footnotes
Contributors WAW: data collection, data analysis and drafting the article; LEC: drafting of the article; DP and AC: planning of the project and critical revision of the article; DPD: planning of the project, data analysis and critical revision of the article; PN, data analysis; DRT: conception of the project, supervision of the project and critical revision of the article.
Funding DRT was funded by the Medical Research Council. LEC is funded by a National Institute for Health Research Academic Clinical Fellowship. These funders had no role or involvement in this study.
Competing interests WAW received an unrestricted educational grant from Boehringer Ingelheim to attend a national meeting. This was awarded after the research was completed. There were no other potential competing interests for any of the authors.
Patient consent for publication Not required.
Ethics approval Only data that were obtained as part of routine clinical care were collected and entered into the interstitial lung disease audit. All data were deidentified and entered by the local clinical care team without linkage to any patient identifiers in line with national and local guidance. The Health Research Authority guidance was followed, and therefore patient consent and ethical approval was not required. Approval for this project was obtained from the University Hospitals Birmingham Clinical Audit Department. This study was conducted according to the principles of the World Medical Association Declaration of Helsinki.
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement Data are available upon reasonable request. Deidentified patient database was used to generate the results of this paper. Database includes an anonymised list of patients with idiopathic pulmonary fibrosis at Queen Elizabeth Hospital, Birmingham, with demographics and various data including lung function and mortality. Available on request from WW (will.a.wright9@gmail.com).
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