LONG-TERM EFFECTIVENESS OF ANTIFIBROTIC DRUGS IN PULMONARY FIBROSIS
Abstract
Pulmonary fibrosis (PF) is a progressive and life-threatening lung disease characterized by excessive deposition of extracellular matrix, leading to irreversible scarring and impaired respiratory function. Antifibrotic drugs, such as pirfenidone and nintedanib, have emerged as standard therapies aimed at slowing disease progression. Recent studies demonstrate that long-term use of these agents can reduce the decline in lung function, decrease hospitalization rates, and improve overall survival [1,2]. Despite these benefits, challenges remain, including adverse effects, patient adherence, and variability in therapeutic response. Understanding the long-term effectiveness and safety profile of antifibrotic therapy is crucial for optimizing patient management and improving quality of life in PF patients [3,4].
Keywords
pulmonary fibrosis, antifibrotic drugs, pirfenidone, nintedanib, long-term efficacy, lung function.
References
- Pacheco Miranda FJ, Vargas Gomez J, Villanueva Guerrero VR, et al. Comparative LongTerm Effectiveness of Nintedanib and Pirfenidone in Idiopathic Pulmonary Fibrosis: A Systematic Literature Review. Journal of Pulmonology Research & Reports. 2025. This review highlights longterm efficacy and tolerability of antifibrotic agents, demonstrating sustained benefits in reducing FVC decline and similar effectiveness between drugs.
- Lalla F, Latella A, Varone F, et al. Longterm treatment with nintedanib and pirfenidone in idiopathic pulmonary fibrosis: a comparative, realworld cohort study. BMC Pulmonary Medicine. 2025;25:455. This realworld cohort confirms comparable longterm efficacy of antifibrotic therapies with manageable safety profiles.
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- Cameli P, d’Alessandro M, et al. Functional and survival outcomes following antifibrotic therapy in IPF. Frontiers in Molecular Biosciences. 2020. Reallife followup demonstrating stabilization of lung function and survival.
- Rochwerg B, et al. Metaanalysis comparing outcomes with pirfenidone and nintedanib, suggesting similar survival benefits and FVC decline slowing.
- Cerri S, et al. Attenuation of DLCO decline with antifibrotic therapy. Observational data supporting functional benefits.
- Maher TM, et al. Impact of dose reduction on nintedanib efficacy. Subgroup analysis from clinical trials showing similar outcomes with adjusted doses.
- Metaanalyses of antifibrotic agents versus placebo show reduced lung function decline and mortality relative to controls.
- Consensus guidelines emphasize early initiation of antifibrotic therapy to maximize patient outcomes in PF.
- Realworld cohort evidence suggests longterm antifibrotic therapy reduces hospitalization and acute exacerbations.
- Observational data on tolerability profiles highlights manageable side effects with both pirfenidone and nintedanib.
- Multiple clinical studies show antifibrotic therapy improves functional metrics such as 6minute walk test and qualityoflife scores.