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      Efectividad y seguridad de pirfenidona en la fibrosis pulmonar idiopática Translated title: Effectiveness and safety of pirfenidone in idiopathic pulmonary fibrosis

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          Abstract

          Resumen Objetivo: La fibrosis pulmonar idiopática (FPI) es una enfermedad caracterizada por una cicatrización progresiva de los pulmones provocando su deterioro a largo plazo. Su diagnóstico requiere la existencia del patrón radiológico denominado neumonía intersticial usual (NIU). Su prevalencia es mayor en hombres. Entre los síntomas principales encontramos la disnea y la tos. En la actualidad, no existe una cura conocida, sin embargo, existen diversos tratamientos antifibróticos enfocados a disminuir la progresión de esta patología y prolongar la supervivencia. El objetivo es evaluar la efectividad y seguridad de la pirfenidona en esta enfermedad. Métodos: Estudio descriptivo retrospectivo entre enero de 2016 y diciembre de 2019. Se incluyeron 40 pacientes diagnosticados de fibrosis pulmonar idiopática que iniciaron tratamiento con pirfenidona. Resultados: Los parámetros de función pulmonar mostraron valores mantenidos durante el periodo de estudio. Hubo mejoría de la tos. Las reacciones adversas más comunes fueron alteraciones gastrointestinales y cutáneas. Conclusión: El tratamiento con pirfenidona requiere de un riguroso seguimiento por el porcentaje de pacientes que abandonó esta terapia.

          Translated abstract

          Abstract Objective: Idiopathic pulmonary fibrosis (IPF) is a disease characterized by progressive scarring of the lungs causing their long-term deterioration. Its diagnosis requires the existence of the radiological pattern called usual interstitial pneumonia (UIP). Its prevalence is higher in men. Among the main symptoms, we find dyspnea and cough. At present, there is no known cure, however, there are various antifibrotic treatments focused on slowing the progression of this pathology and prolonging survival. The aim of this study was to evaluate the effectiveness and safety of pirfenidone in this disease. Methods: Retrospective descriptive study from January 2016 to December 2019. The study included 40 patients diagnosed with idiopathic pulmonary fibrosis who started treatment with pirfenidone. Results: The pulmonary function parameters showed values that were maintained during the study period. There was an improvement in the cough. The most common adverse reactions were gastrointestinal and skin disorders. Conclusion: Pirfenidone treatment requires rigorous monitoring due to the percentage of patients who abandoned this therapy.

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          Effect of continued treatment with pirfenidone following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis

          Background The assessment of treatment response in idiopathic pulmonary fibrosis (IPF) is complicated by the variable clinical course. We examined the variability in the rate of disease progression and evaluated the effect of continued treatment with pirfenidone in patients who experienced meaningful progression during treatment. Methods The source population included patients enrolled in the ASCEND and CAPACITY trials (N=1247). Pearson's correlation coefficients were used to characterise the relationship between changes in FVC during consecutive 6-month intervals in the placebo population. Outcomes following a ≥10% decline in FVC were evaluated by comparing the proportion of patients in the pirfenidone and placebo groups who experienced a ≥10% decline in FVC or death during the subsequent 6 months. Results A weak negative correlation was observed between FVC changes during consecutive intervals in the placebo population (coefficient, −0.146, p<0.001), indicating substantial variability. Thirty-four (5.5%) and 68 (10.9%) patients in the pirfenidone and placebo groups, respectively, experienced a ≥10% decline in FVC by month 6. During the subsequent 6 months, fewer patients in the pirfenidone group compared with placebo experienced a ≥10% decline in FVC or death (5.9% vs 27.9%; relative difference, 78.9%). There was one (2.9%) death in the pirfenidone group and 14 (20.6%) deaths in the placebo group (relative difference, 85.7%). Conclusions Longitudinal FVC data from patients with IPF showed substantial intrasubject variability, underscoring the inability to reliably assess therapeutic response using serial FVC trends. In patients who progressed during treatment, continued treatment with pirfenidone resulted in a lower risk of subsequent FVC decline or death. Trial registration numbers NCT01366209, NCT00287729, NCT00287716.
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            Real World Experiences: Pirfenidone and Nintedanib are Effective and Well Tolerated Treatments for Idiopathic Pulmonary Fibrosis

            Idiopathic Pulmonary Fibrosis (IPF) now has two licensed treatments available. Pirfenidone was the first drug to be licensed and approved for use, followed by nintedanib. We set out our real world experience with these agents in terms of their adverse events profile outside the restrictions of a clinical trial. We have demonstrated in the real world setting, that side effects are common and predominantly gastrointestinal with both therapies. Our study shows that the side effects can be effectively managed in the majority of patients with an acceptable discontinuation rate similar to that seen in the clinical trials. These findings are compelling despite the fact that the patients in our study are older, have severer disease as depicted by baseline lung function and more co-morbidities. Our data provides ongoing evidence of the safety and tolerability of both pirfenidone and nintedanib in patients who would not have met the rigorous criteria to be included in a clinical trial. Both these agents are effective in the management of IPF and slow the progression of this debilitating life limiting condition.
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              Pirfenidone, nintedanib and N-acetylcysteine for the treatment of idiopathic pulmonary fibrosis: A systematic review and meta-analysis.

              The prevalence of idiopathic pulmonary fibrosis (IPF) is increasing every year. Pirfenidone and nintedanib were approved for treatment of IPF in 2014, but they received only a conditional recommendation for use and, thus, to date no drugs are strongly recommended for IPF. The aim of this study was to assess the effectiveness and safety of the currently approved drugs for IPF and N-acetylcysteine (NAC), the most debated drug in the last update of guidelines for IPF treatment.
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                Author and article information

                Journal
                ofil
                Revista de la OFIL
                Rev. OFIL·ILAPHAR
                Organización de Farmacéuticos Ibero-Latinoamericanos (Madrid, Madrid, Spain )
                1131-9429
                1699-714X
                June 2022
                : 32
                : 2
                : 189-192
                Affiliations
                [1] Pontevedra orgnameComplejo Hospitalario Universitario de Pontevedra orgdiv1Servicio de Farmacia España rosagimenezcandela@ 123456gmail.com
                Article
                S1699-714X2022000200012 S1699-714X(22)03200200012
                10.4321/s1699-714x2022000200012
                bcf4cf48-c47e-4900-aaf3-8c0080a3b347

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 25 November 2020
                : 07 November 2020
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 13, Pages: 4
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                SciELO Spain

                Categories
                Originales

                pirfenidone,Idiopathic pulmonary fibrosis,efectiveness,safety,antifibrotic,Fibrosis pulmonar idiopática,pirfenidona,efectividad,seguridad,antifibrótico

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