Efficacy of Pirfenidone in Patients with Advanced-stage Idiopathic Pulmonary Fibrosis
Open accessAbstract
OBJECTIVE: To assess the efficacy of pirfenidone in patients with advanced-stage idiopathic pulmonary fibrosis (IPF), we conducted a retrospective study of patients who received pirfenidone therapy. In addition, the combined effects of inhaled N-acetylcysteine (NAC) and pirfenidone were evaluated. METHODS: Eligible patients had a clinical and radiologic diagnosis of advanced-stage IPF (stages of severity III&IV). Patients who exhibited a relative decline in forced vital capacity (FVC) of 10% or more within the preceding six (±2) months were enrolled. The outcome was evaluated from the date of the 6-month follow-up PFT. Relative declines in FVC of more than 10% were defined as progressive disease (ineffective group), while those less than 10% were defined as stable disease (effective group). The clinical features were compared between the two groups. We also compared the efficacy of the combined therapy with inhaled NAC and pirfenidone (n=11) with that of pirfenidone alone (n=7). RESULTS: Eighteen patients 59-82 years of age with IPF who received pirfenidone therapy were reviewed. Pirfenidone stabilized declines in FVC by 10% at six months in eight of the 18 cases (44%). The median changes in FVC at six months were +120 mL in the effective group and -590 mL in the ineffective group. The number of NAC users was significantly higher in the effective group (7/8=87.5%) than in the ineffective group (3/10=30%) (p=0.02). Furthermore, the use of combined NAC therapy was correlated with a favorable outcome. The median change in FVC at six months was 0 mL in the NAC group and -290 mL in the non-NAC group. The median survival period was 557 ± 66 days in the NAC group and 196 ± 57 days in the non-NAC group (p=0.03). CONCLUSION: Among the advanced-stage IPF patients with a more progressive status, pirfenidone decreased the rate of decline in FVC. In addition, patients treated with pirfenidone combined with NAC therapy exhibited favorable outcomes. Additional studies are needed to confirm the efficacy of this combined therapy for IPF.
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In the INSTAGE trial of 274 IPF patients, nintedanib plus sildenafil showed no significant benefit compared with nintedanib alone.
“When nintedanib was paired with sildenafil in the 274 IPF patients INSTAGE trial, no significant benefit was seen compared to treatment with nintedanib alone [82].”
From Emerging pharmacological options in the treatment of idiopathic pulmonary fibrosis (IPF) (2024) · cites this paper as [82]
The Abstract describes a retrospective study of 18 IPF patients receiving pirfenidone, including a comparison of pirfenidone with and without inhaled NAC. Searches for INSTAGE, 274 patients, nintedanib, and sildenafil found no passage addressing the claimed trial or its result. (LLM, conf=0.78)
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Raghu, G., Collard, H. R., Egan, J. J., Martinez, F. J., Behr, J., Brown, K. K., Colby, T. V., Cordier, J.-F., Flaherty, K. R., Lasky, J. A., et al. (2011) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.2009-040gl · Cited by 7,186 · see citing works
Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials
Noble, P. W., Albera, C., Bradford, W. Z., Costabel, U., Glassberg, M. K., Kardatzke, D., King, T. E., Lancaster, L., Sahn, S. A., Szwarcberg, J., et al. (2011) · The Lancet · doi:10.1016/s0140-6736(11)60405-4 · Cited by 2,154 · see citing works
High-Dose Acetylcysteine in Idiopathic Pulmonary Fibrosis
Demedts, M., Behr, J., Buhl, R., Costabel, U., Dekhuijzen, R., Jansen, H. M., MacNee, W., Thomeer, M., Wallaert, B., Laurent, F., et al. (2005) · New England Journal of Medicine · doi:10.1056/nejmoa042976 · Cited by 964 · see citing works
Pirfenidone in idiopathic pulmonary fibrosis
Taniguchi, H., Ebina, M., Kondoh, Y., Ogura, T., Azuma, A., Suga, M., Taguchi, Y., Takahashi, H., Nakata, K., Sato, A., et al. (2009) · European Respiratory Journal · doi:10.1183/09031936.00005209 · Cited by 957 · see citing works
Double-blind, Placebo-controlled Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis
Azuma, A., Nukiwa, T., Tsuboi, E., Suga, M., Abe, S., Nakata, K., Taguchi, Y., Nagai, S., Itoh, H., Ohi, M., et al. (2005) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200404-571oc · Cited by 953 · see citing works
Pirfenidone in idiopathic pulmonary fibrosis: Figure 1–
Swigris, J., & Fairclough, D. (2010) · European Respiratory Journal · doi:10.1183/09031936.00071710 · Cited by 802 · see citing works
BUILD-3: A Randomized, Controlled Trial of Bosentan in Idiopathic Pulmonary Fibrosis
King, T. E., Brown, K. K., Raghu, G., du Bois, R. M., Lynch, D. A., Martinez, F., Valeyre, D., Leconte, I., Morganti, A., Roux, S., et al. (2011) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201011-1874oc · Cited by 718 · see citing works
A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis
Raghu, G., Brown, K. K., Bradford, W. Z., Starko, K., Noble, P. W., Schwartz, D. A., & King, T. E. (2004) · New England Journal of Medicine · doi:10.1056/nejmoa030511 · Cited by 652 · see citing works
King, T. E., Albera, C., Bradford, W. Z., Costabel, U., Hormel, P., Lancaster, L., Noble, P. W., Sahn, S. A., Szwarcberg, J., Thomeer, M., et al. (2009) · The Lancet · doi:10.1016/s0140-6736(09)60551-1 · Cited by 531 · see citing works
Imatinib Treatment for Idiopathic Pulmonary Fibrosis: Randomized Placebo-controlled Trial Results
Daniels, C. E., Lasky, J. A., Limper, A. H., Mieras, K., Gabor, E., & Schroeder, D. R. (2010) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200906-0964oc · Cited by 373 · see citing works
Zappala, C. J., Latsi, P. I., Nicholson, A. G., Colby, T. V., Cramer, D., Renzoni, E. A., Hansell, D. M., du Bois, R. M., & Wells, A. U. (2009) · European Respiratory Journal · doi:10.1183/09031936.00155108 · Cited by 371 · see citing works
Hagiwara, S.-I., Ishii, Y., & Kitamura, S. (2000) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.162.1.9903129 · Cited by 219 · see citing works
Homma, S. A., Azuma, A., Taniguchi, H., Ogura, T., Mochiduki, Y., Sugiyama, Y., Nakata, K., Yoshimura, K., Takeuchi, M., & Kudoh, S. (2012) · Respirology · doi:10.1111/j.1440-1843.2012.02132.x · Cited by 127 · see citing works
Self-citationTaniguchi, H., Kondoh, Y., Ebina, M., Azuma, A., Ogura, T., Taguchi, Y., Suga, M., Takahashi, H., Nakata, K., Sato, A., et al. (2011) · Respiratory Research · doi:10.1186/1465-9921-12-93 · Cited by 78 · see citing works
Muramatsu, Y., Sugino, K., Kikuchi, N., et al. (2011) · doi:10.1164/ajrccm-conference.2011.183.1_meetingabstracts.a1529 · Cited by 4 · see citing works
Self-citationIshida, F., Sugino, K., Muramatsu, Y., et al. (2009) · doi:10.1164/ajrccm-conference.2009.179.1_meetingabstracts.a4154 · Cited by 2 · see citing works
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Rogliani, P., Calzetta, L., Cavalli, F., Matera, M. G., & Cazzola, M. (2016) · Pulmonary Pharmacology & Therapeutics · doi:10.1016/j.pupt.2016.07.009 · Cited by 168 · see citing works
Emerging pharmacological options in the treatment of idiopathic pulmonary fibrosis (IPF)
Aribindi, K., Liu, G. Y., & Albertson, T. E. (2024) · Expert Review of Clinical Pharmacology · doi:10.1080/17512433.2024.2396121 · Cited by 17 · see citing works
Zhang, X.-L., Cao, Y., & Zheng, B. (2023) · BMC Pulmonary Medicine · doi:10.1186/s12890-023-02778-w · Cited by 7 · see citing works
- Authors
- Sakamoto, S., Itoh, T., Muramatsu, Y., Satoh, K., Ishida, F., Sugino, K., Isobe, K., & Homma, S.
- Affiliations
- Toho University Omori Medical Center (JP)
- Venue
- Internal Medicine
- Published
- 2013
- Volume
- 52
- Issue
- 22
- Pages
- 2495-2501
- ISSN
- 0918-2918 (ISSN-L)
- 1349-7235
- OpenAlex
- W2018381516
- Cited by
- 59
- References
- 16
- Citations by year
- 2025: 2 · 2024: 18 · 2023: 2 · 2021: 2 · 2020: 2 · 2019: 7 · 2018: 2 · 2017: 8 · 2016: 4 · 2015: 8 · 2014: 3 · 2013: 1
- FWCI
- 4.33
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Internal Medicine (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Chronic Obstructive Pulmonary Disease (COPD) Research
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
- Keywords
- pirfenidone, idiopathic pulmonary fibrosis, N-acetylcysteine, combination therapy, forced vital capacity decline, disease progression, survival, antifibrotic therapy
- MeSH terms
- Acetylcysteine, Administration, Inhalation, Aged, Aged, 80 and over, Anti-Inflammatory Agents, Non-Steroidal, Antioxidants, Drug Therapy, Combination, Female, Humans, Male, Middle Aged, Pyridones, Retrospective Studies, Vital Capacity, Treatment Outcome, Disease Progression, Idiopathic Pulmonary Fibrosis
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