Efficacy of pirfenidone for idiopathic pulmonary fibrosis: An Italian real life study
Open accessAbstract
Background In this retrospective Italian study, which involved all major national interstitial lung diseases centers, we evaluated the effect of pirfenidone on disease progression in patients with IPF.Methods We retrospectively studied 128 patients diagnosed with mild, moderate or severe IPF, and the decline in lung function monitored during the one-year treatment with pirfenidone was compared with the decline measured during the one-year pre-treatment period.Results At baseline (first pirfenidone prescription), the mean percentage forced vital capacity (FVC) was 75% (35-143%) of predicted, and the mean percentage diffuse lung capacity (DLCO) was 47% (17-120%) of predicted. Forty-eight patients (37.5%) had mild disease (GAP index stage I), 64 patients (50%) had moderate IPF (stage II), and 8 patients (6.3%) had severe disease (stage III). In the whole population, pirfenidone attenuated the decline in FVC (p = 0.065), but did not influence the decline in DLCO (p = 0.355) in comparison to the pre-treatment period. Stratification of patients into mild and severe disease groups based on %FVC level at baseline (>75% and ≤75%) revealed that attenuation of decline in FVC (p = 0.002) was more pronounced in second group of patients. Stratification of patients according to GAP index at baseline (stage I vs. II/III) also revealed that attenuation of decline in lung function was more pronounced in patients with more severe disease.Conclusions In this national experience, pirfenidone reduced the rate of annual FVC decline (p = 0.065). Since pirfenidone provided significant treatment benefit for patients with moderate-severe disease, our results suggest that the drug may also be effective in patients with more advanced disease.
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Systematic reviews and meta-analyses failed to show any therapeutic benefit of systemic corticosteroids in IPF.
“Systemic corticosteroids have been widely studied in the treatment of IPF with systematic reviews and meta-analyses failing to show any therapeutic benefit [23,52,53].”
From Emerging pharmacological options in the treatment of idiopathic pulmonary fibrosis (IPF) (2024) · cites this paper as [23]
The Discussion (p. 8) says corticosteroids have been demonstrated ineffective in IPF, and the References (p. 9) include a systematic review on corticosteroids. The paper does not say that multiple systematic reviews and meta-analyses reached this conclusion; its own study evaluated pirfenidone, not corticosteroids. (LLM, conf=0.84)
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- Authors
- Harari, S., Caminati, A., Albera, C., Vancheri, C., Poletti, V., Pesci, A., Luppi, F., Saltini, C., Agostini, C., Bargagli, E., Sebastiani, A., Sanduzzi, A., Giunta, V., Della Porta, R., Bandelli, G. P., Puglisi, S., Tomassetti, S., Biffi, A., Cerri, S., Mari, A., Cinetto, F., Tirelli, F., Farinelli, G., Bocchino, M., Specchia, C., & Confalonieri, M.
- Affiliations
- MultiMedica (IT)
- University of Turin (IT)
- University of Catania (IT)
- Ospedale G.B. Morgagni - L.Pierantoni (IT)
- University of Milano-Bicocca (IT)
- Azienda Ospedaliero-Universitaria di Modena (IT)
- Policlinico Tor Vergata (IT)
- University of Padua (IT)
- University of Siena (IT)
- Azienda Ospedaliera San Camillo-Forlanini (IT)
- Ospedale Monaldi (IT)
- University of Trieste (IT)
- Corresponding author
- Harari, S.
- ORCID
- Venue
- Respiratory Medicine
- Publisher
- Elsevier BV
- Published
- 2015-07
- Volume
- 109
- Issue
- 7
- Pages
- 904-913
- ISSN
- 0954-6111 (ISSN-L)
- OpenAlex
- W2045756883
- Cited by
- 78
- References
- 23
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 2 · 2023: 1 · 2022: 3 · 2021: 5 · 2020: 10 · 2019: 13 · 2018: 13 · 2017: 14 · 2016: 9 · 2015: 4 · 2013: 1
- FWCI
- 10.56
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Respiratory Medicine (publishedVersion)
- PubMed (publishedVersion)
- ArTS Archivio della ricerca di Trieste (University of Trieste https://www.units.it/) (submittedVersion, other-oa)
- Institutional Research Information System University of Turin (University of Turin) (submittedVersion, other-oa)
- Archivio Istituzionale della Ricerca (Universita Degli Studi Di Milano) (submittedVersion, other-oa)
- BOA (University of Milano-Bicocca) (submittedVersion, other-oa)
- Institutional Research Information System (Università degli Studi di Brescia) (submittedVersion)
- IRIS UNIMORE (University of Modena and Reggio Emilia) (submittedVersion, cc-by)
- Use Siena air (University of Siena) (submittedVersion, other-oa)
- Padua Research Archive (University of Padova) (submittedVersion)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
- Keywords
- pirfenidone, idiopathic pulmonary fibrosis, lung function decline, forced vital capacity, diffusing capacity for carbon monoxide, GAP index, disease severity, real-world effectiveness, antifibrotic therapy
- MeSH terms
- Aged, Anti-Inflammatory Agents, Non-Steroidal, Female, Humans, Italy, Male, Pyridones, Retrospective Studies, Vital Capacity, Incidence, Treatment Outcome, Disease Progression, Idiopathic Pulmonary Fibrosis
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