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Article

Real-World Clinical Effectiveness and Safety of Antifibrotics in Progressive Pulmonary Fibrosis Associated with Rheumatoid Arthritis

by
Javier Narváez
1,*,†,
Martí Aguilar-Coll
1,†,
Vanesa Vicens-Zygmunt
2,
Juan José Alegre
3,
Guadalupe Bermudo
2 and
María Molina-Molina
2
1
Department of Rheumatology, Hospital Universitario de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), 08907 Barcelona, Spain
2
Interstitial Lung Disease Unit, Department of Pneumology, Hospital Universitario de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), 08907 Barcelona, Spain
3
Department of Rheumatology, Hospital Universitario Dr. Peset, 46017 Valencia, Spain
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
J. Clin. Med. 2024, 13(23), 7074; https://doi.org/10.3390/jcm13237074
Submission received: 4 November 2024 / Revised: 21 November 2024 / Accepted: 21 November 2024 / Published: 22 November 2024
(This article belongs to the Special Issue Rheumatoid Arthritis: Clinical Updates on Diagnosis and Treatment)

Abstract

Background/Objectives: Interstitial lung disease (ILD) is one of the most severe complications of rheumatoid arthritis (RA). Real-world data on antifibrotic treatment are needed. Our objective was to evaluate the real-world effectiveness and tolerability of antifibrotic agents in patients with progressive fibrosing RA-ILD. Methods: A longitudinal, retrospective, observational study was conducted on a cohort of RA-ILD patients treated with either nintedanib or pirfenidone. The data collected included pulmonary function test (PFT) results, adverse events (AEs), tolerability, and drug retention. Results: Twenty-seven patients were included; 25 (92.5%) initiated nintedanib, while two initiated pirfenidone. The median follow-up duration was 25 months (IQR 7–27). The mean decline in %pFVC and %pDLCO from ILD diagnosis to the initiation of antifibrotic therapy were −8.9% and −14.8%, respectively. After 6 months of treatment, most patients achieved stabilization in PFT: a ∆%pFVC of +1.2% (p = 0.611 compared with baseline) and a ∆%pDLCO of +3.9% (p = 0.400). Eighteen patients completed one year of therapy, with a modest improvement in %pFVC (+4.7%; p = 0.023) and stabilization in %pDLCO (−3.8%; p = 0.175). This trend persisted among the nine patients who completed 2 years of treatment (%pFVC +7.7%; p = 0.037 and %pDLCO −2.2%; p = 0.621). During the follow-up period, 15% of patients died, and 4% underwent lung transplantation. Adverse events occurred in 81% of patients, leading to discontinuation in 18.5% of cases. The most frequent adverse events were gastrointestinal events and hepatitis, leading to a permanent dose reduction of 40% for nintedanib and 14% for pirfenidone. A second antifibrotic agent was prescribed for 18.5% of the patients. At the end of the follow-up period, 63% of the total cohort remained on antifibrotic therapy. Conclusions: According to our results, antifibrotic initiation was associated with a modest improvement in the trajectory of %pFVC and stabilization in %pDLCO. The discontinuation rate in our cohort (37%) was higher than that reported in clinical trials but similar to that reported in previously published real-world studies.
Keywords: rheumatoid arthritis; interstitial lung diseases; antifibrotics; nintedanib; pirfenidone rheumatoid arthritis; interstitial lung diseases; antifibrotics; nintedanib; pirfenidone

Share and Cite

MDPI and ACS Style

Narváez, J.; Aguilar-Coll, M.; Vicens-Zygmunt, V.; Alegre, J.J.; Bermudo, G.; Molina-Molina, M. Real-World Clinical Effectiveness and Safety of Antifibrotics in Progressive Pulmonary Fibrosis Associated with Rheumatoid Arthritis. J. Clin. Med. 2024, 13, 7074. https://doi.org/10.3390/jcm13237074

AMA Style

Narváez J, Aguilar-Coll M, Vicens-Zygmunt V, Alegre JJ, Bermudo G, Molina-Molina M. Real-World Clinical Effectiveness and Safety of Antifibrotics in Progressive Pulmonary Fibrosis Associated with Rheumatoid Arthritis. Journal of Clinical Medicine. 2024; 13(23):7074. https://doi.org/10.3390/jcm13237074

Chicago/Turabian Style

Narváez, Javier, Martí Aguilar-Coll, Vanesa Vicens-Zygmunt, Juan José Alegre, Guadalupe Bermudo, and María Molina-Molina. 2024. "Real-World Clinical Effectiveness and Safety of Antifibrotics in Progressive Pulmonary Fibrosis Associated with Rheumatoid Arthritis" Journal of Clinical Medicine 13, no. 23: 7074. https://doi.org/10.3390/jcm13237074

APA Style

Narváez, J., Aguilar-Coll, M., Vicens-Zygmunt, V., Alegre, J. J., Bermudo, G., & Molina-Molina, M. (2024). Real-World Clinical Effectiveness and Safety of Antifibrotics in Progressive Pulmonary Fibrosis Associated with Rheumatoid Arthritis. Journal of Clinical Medicine, 13(23), 7074. https://doi.org/10.3390/jcm13237074

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