Efficacy and Tolerability Outcomes of Treatment for Idiopathic Pulmonary Fibrosis. Real-life Experience

Main Article Content

Raquel López Vime
https://orcid.org/0000-0001-7081-1341
María José Pavón Fernández
https://orcid.org/0000-0003-1726-9922
Susana Flórez Martín
https://orcid.org/0000-0001-9762-9195
Rosa Rojo López

Abstract

Introduction: idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease with a very poor prognosis. Two drugs are currently available for this disease. The purpose of our study is to evaluate the effects of treatment in patients in a real-life practice.


Methods: retrospective cohort study, without a control group. Patients attending a specific pulmonary fibrosis consultation between January 2015 and May 2021 were collected. We assessed the evolution of lung function before and after initiation of antifibrotic treatment, degree of dyspnea, side effects and mortality. 


Results: fifty-seven patients were included, 45 received pirfenidone and 12 nintedanib. Eighty-two per cent were male and the mean age was 76 years. Follow-up was 30 months. The most frequent diagnostic method was high-resolution computed axial tomography (HRCT) in 58%. The most frequent side effects were digestive (38.6%). There was no drop in the forced vital capacity (FVC) above 10% at any time during follow-up, although a worsening of the degree of dyspnea was observed.  During follow-up, 45.6% of patients died. 


Conclusion: our results show that both pirfenidone and nintedanib are well tolerated in most patients with IPF. We observed a stability of pulmonary function during the follow-up of the surviving patients.

Downloads

Download data is not yet available.

Article Details

How to Cite
López Vime, R., Pavón Fernández, M. J., Flórez Martín, S., & Rojo López, R. (2023). Efficacy and Tolerability Outcomes of Treatment for Idiopathic Pulmonary Fibrosis. Real-life Experience. Respirar, 15(3). https://doi.org/10.55720/respirar.15.3.2
Section
Artículos Originales

References

American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 2000;161(2 Pt 1):646-64. Doi: 10.1164/ajrccm.161.2.ats3-00

King TE, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet 2011;378(9807):1949-61. Doi: 10.1016/S0140-6736(11)60052-4.

Cancer Facts & Figures 2019. American Cancer Society [Internet] [Consultado 29 mar 2023]. Disponible en: https://www.cancer.org/research/cancer-facts-statistics/all-cancer-facts-figures/cancer-facts-figures-2019.html

Ley B, Collard HR, King TE. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011;183(4):431-40. Doi: 10.1164/rccm.201006-0894CI.

Ley B, J. Ryerson C, Vittinghoff E et al. A Multidimensional Index and Staging System for Idiopathic Pulmonary Fibrosis. Ann Intern Med 2012. Doi: 10.7326/0003-4819-156-10-201205150-00004

Noble PW, Albera C, Bradford WZ et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011;377(9779):1760-9. Doi: 10.1016/S0140-6736(11)60405-4.

Richeldi L, du Bois RM, Raghu G et al. Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis. N Engl J Med 2014;370(22):2071-82. Doi: 10.1056/NEJMoa1402584.

Ministerio de Sanidad. Agencia Española del Medicamento y Productos Sanitarios. Informe de posicionamiento terapéutico de nintedanib.IPT:07/2022 [Internet]. [Consultado 1 Feb 2023] Disponible en: https://www.aemps.gob.es/medicamentosUsoHumano/informesPublicos/docs/2022/IPT_07-2022-nintedanib.pdf

Ministerio de Sanidad. Agencia Española del Medicamento y Productos Sanitarios. Informe de posicionamiento terapéutico de pirfenidona.IPT:53/2016 [Internet]. [Consultado 1 Feb 2023] Disponible en: https://www.aemps.gob.es/medicamentosUsoHumano/informesPublicos/dpcs/IPT-pirfenidona-Esbriet-FPI.pdf

Bargagli E, Piccioli C, Rosi E et al. Pirfenidone and Nintedanib in idiopathic pulmonary fibrosis: Real-life experience in an Italian referral centre. Pulmonology 2019;25(3):149-53. Doi: 10.1016/j.pulmoe.2018.06.003.

Cerri S, Monari M, Guerrieri A et al. Real-life comparison of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis: A 24-month assessment. Respir Med 2019;159:105803. Doi: 10.1016/j.rmed.2019.105803.

Rochwerg B, Neupane B, Zhang Y et al. Treatment of idiopathic pulmonary fibrosis: a network meta-analysis. BMC Med 2016;14:18. Doi: 10.1186/s12916-016-0558-x.

King TE, Bradford WZ, Castro-Bernardini S et al. A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis. N Engl J Med. 29 de mayo de 2014;370(22):2083-92. Doi: 10.1056/NEJMoa1402582

Behr J, Prasse A, Wirtz H et al. Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry. Eur Respir J 2020;56(2):1902279. Doi: 10.1183/13993003.02279-2019.

Kreuter M. Pirfenidone: an update on clinical trial data and insights from everyday practice. Eur Respir Rev 2014;23(131):111-7. Doi: 10.1183/09059180.00008513

Vietri L, Cameli P, Perruzza M et al. Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena. Ther Adv Respir Dis 2020;14:1753466620906326. Doi: 10.1177/1753466620906326

Harari S, Caminati A, Poletti V et al. A Real-Life Multicenter National Study on Nintedanib in Severe Idiopathic Pulmonary Fibrosis. Respir Int Rev Thorac Dis 2018;95(6):433-40. Doi: 10.1159/000487711.

Wuyts WA, Dahlqvist C, Slabbynck H et al. Quality of Life and Healthcare Resource Use in a Real-world Patient Population with Idiopathic Pulmonary Fibrosis: The PROOF Registry. Pulm Ther 2022;8(2):181-94. Doi: 10.1007/s41030-022-00187-8

Raghu G, Remy-Jardin M, Myers JL et al. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med 2018;198(5):e44-68. Doi: 10.1164/rccm.201807-1255ST.

Raghu G, Remy-Jardin M, Richeldi L et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med 2022;205(9):e18-47. Doi: 10.1164/rccm.202202-0399ST.

Normativas SEPAR [Internet]. [Consultado 21 de noviembre de 2022]. Disponible en: https://www.separcontenidos.es/normativasSEPAR/index.php?frameInferior=publicacionesbis&ID_Normativa=24&Admin=0&Idioma=Esp&ID_MenuPublicaciones=

Tran T, Šterclová M, Mogulkoc N et al. The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis. Respir Res 2020;21(1):11. Doi: 10.1186/s12931-019-1271-z.

Fernández-Fabrellas E, Molina-Molina M, Soriano JB et al. Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry. Respir Res 2019;20(1):127. Doi: 10.1186/s12931-019-1084-0

Raghu G, Collard HR, Egan JJ et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183(6):788-824. Doi: 10.1164/rccm.2009-040GL

Cottin V, Koschel D, Günther A et al. Long-term safety of pirfenidone: results of the prospective, observational PASSPORT study. ERJ Open Res 2018;4(4):00084-2018. Doi: 10.1183/23120541.00084-2018

Skandamis A, Kani C, Markantonis SL, Souliotis K. Systematic review and network meta-analysis of approved medicines for the treatment of idiopathic pulmonary fibrosis. J Drug Assess 2019;8(1):55-61. Doi: 10.1080/21556660.2019.1597726

Oltmanns U, Kahn N, Palmowski K, Träger A, Wenz H, Heussel CP, et al. Pirfenidone in Idiopathic Pulmonary Fibrosis: Real-Life Experience from a German Tertiary Referral Center for Interstitial Lung Diseases. Respiration 2014;88(3):199-207. Doi: 10.1159/000363064.

Corral M, DeYoung K, Kong AM. Treatment patterns, healthcare resource utilization, and costs among patients with idiopathic pulmonary fibrosis treated with antifibrotic medications in US-based commercial and Medicare Supplemental claims databases: a retrospective cohort study. BMC Pulm Med 2020;20(1):188. Doi: 10.1186/s12890-020-01224-5.

Harari S, Caminati A. Idiopathic pulmonary fibrosis: from clinical trials to real-life experiences. Eur Respir Rev Off J Eur Respir Soc 2015;24(137):420-7. Doi: 10.1183/16000617.0042-2015.

Cameli P, Refini RM, Bergantini L et al. Long-Term Follow-Up of Patients With Idiopathic Pulmonary Fibrosis Treated With Pirfenidone or Nintedanib: A Real-Life Comparison Study. Front Mol Biosci 2020;7:581828. Doi: 10.3389/fmolb.2020.581828