Pirfenidone and Nintedanib in idiopathic pulmonary fibrosis: Real-life experience in an Italian referral centre

Bargagli, Elena; Piccioli, Caterina; Rosi, Elisabetta; Torricelli, Elena; Turi, Lorenzo; E, Piccioli; Pistolesi, Massimo; Ferrari, Katia; Voltolini, Luca

doi:10.1016/j.pulmoe.2018.06.003

Cited by 50 publications

(45 citation statements)

References 17 publications

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“…9 Phase III clinical trials ASCEND, CAPACITY 004 and CAPACITY 006 were the first to report a significant beneficial effect of pirfenidone in reducing the decline of forced vital capacity (FVC) in patients with IPF. 10,11 Subsequent open-label extension trials and real-life multicentre studies have confirmed the efficacy of pirfenidone in reducing disease progression, [12][13][14][15][16][17] leading to a significant improvement in survival. 18,19 Safety concerns are few, including specifically gastrointestinal symptoms and photosensitivity, and can be usually resolved or mitigated through a careful management of drug assumption.…”

Section: Introductionmentioning

confidence: 97%

Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena

Vietri

Cameli

Perruzza

et al. 2020

Ther Adv Respir Dis

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Background: Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and has a median survival after diagnosis of 2–5 years. Pirfenidone is the first approved antifibrotic drug for the treatment of IPF. Here we report the functional progress, side effects and survival data of a population of patients with IPF, diagnosed at our centre and treated with pirfenidone. Methods: We enrolled 91 patients with IPF (71 males) treated with pirfenidone. Clinical, survival and functional details were collected retrospectively at start of therapy and after 12, 24, 36 and 48 months of treatment. Lung function tests at least 12 months before starting therapy were available for 40 patients and were entered in the database, as well as side effects. Results: During the observation period (922 ± 529 days), 27 patients died, 5 patients underwent lung transplant and 10 patients interrupted therapy due to adverse events or IPF progression. The median survival was 1606 days. There was a significant reduction in disease progression rate, as measured by trend of forced vital capacity, after 1 year of therapy with respect to before treatment ( p = 0.0085). Forced vital capacity reduction rate was progressively higher in the subsequent years of treatment. Treatment-related side effects were reported in 25 patients and were predominantly mild. Overall, four patients discontinued therapy due to severe photosensitivity. Conclusions: Our findings confirm the efficacy of pirfenidone in reducing functional progression of IPF and its excellent safety profile in a real-life setting. This study, designed on a long-term follow up, contributes to the growing evidence on safety, tolerability and efficacy of pirfenidone in IPF. The reviews of this paper are available via the supplemental material section.

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Section: Introductionmentioning

confidence: 97%

Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena

Vietri

Cameli

Perruzza

et al. 2020

Ther Adv Respir Dis

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“…Published IPF registry studies have provided insights into natural history, patient demographics, diagnostic evaluation practices, quality of life, comorbidities and health care utilization [3][4][5][6][7]. Real world experiences with anti-fibrotic use have also been reported for a single Italian center [8] and in England using administrative data [9]. Additionally, surveys have been used to explore patient and provider views about IPF treatment in Canada, France, Germany, Italy, Spain and the United Kingdom [10].…”

Section: Introductionmentioning

confidence: 99%

Patient and site characteristics associated with pirfenidone and nintedanib use in the United States; an analysis of idiopathic pulmonary fibrosis patients enrolled in the Pulmonary Fibrosis Foundation Patient Registry

Holtze

Freiheit

Limb³

et al. 2020

Respir Res

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Background: Pragmatic use of the anti-fibrotic medications pirfenidone and nintedanib for idiopathic pulmonary fibrosis (IPF) in the United States (US) has not been studied and may be different from international settings due to structural differences between health care systems. This study examined the relationship between patient-and sitelevel characteristics and anti-fibrotic (a) use and (b) selection. Methods: Data from the Pulmonary Fibrosis Foundation Patient Registry was used to perform univariable and multivariable regressions with generalized linear mixed models. A random effects model examined registry site variation. Results: 703 of 1218 (57.7%) patients were taking a single anti-fibrotic of which 312 (44.4%) were taking nintedanib and 391 (55.6%) were taking pirfenidone. Up to 25% of patients using an anti-fibrotic may have been excluded from clinical trial participation due to having too severe disease as measured by diffusion limitation for carbon monoxide. Age (OR = 0.974, p = 0.0086) and diffusion capacity of the lungs for carbon monoxide (per 10% increase in percentpredicted; OR = 0.896, p = 0.0007) was negatively associated with anti-fibrotic use while time (in log of days) since diagnosis (OR = 1.138, p < 0.0001), recent patient clinical trial participation (OR = 1.569, p = 0.0433) and oxygen use (OR = 1.604, p = 0.0027) was positively associated with anti-fibrotic use. Time (log of days) since diagnosis (OR = 1.075, p = 0.0477), history of coronary artery disease (OR = 1.796, p = 0.0030), presence of pulmonary hypertension (OR = 2.139, p = 0.0376), patient clinical trial participation in the prior 12 months (OR = 2.485, p = 0.0002), diffusion capacity of the lungs for carbon monoxide (per 10% increase in percent-predicted; OR = 1.138, p = 0.0184), anticoagulant use (OR = 2.507, p = 0.0028), and enrollment at a registry site in the Midwest region (OR = 1.600, p = 0.0446) were associated with pirfenidone use. Anti-fibrotic use varied by registry site. Rates of discontinuation were modest and nearly identical for the two medications with side effects being the most common reason given for discontinuation. Twenty-three percent (23%, 274) of persons with IPF were using or had recently used an immunomodulatory agent.

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“…Consistent with our findings, data from these studies suggest that nintedanib has a similar safety and tolerability profile in clinical practice as was observed in clinical trials. (16)(17)(18)(19)(20)(21)(22)(23) In post-marketing surveillance data from 6758 patients treated with nintedanib in the US in the year following the launch of nintedanib as a treatment for IPF, diarrhea, nausea and vomiting were the most frequently reported adverse events. (18) In an observational study of 94 patients with IPF in Greece, diarrhea was reported in 55% of patients treated with nintedanib over a follow-up period of 12 months, and 12% of patients discontinued nintedanib due to diarrhea.…”

Section: Discussionmentioning

confidence: 99%

Segurança e tolerabilidade de Nintedanibe em pacientes com fibrose pulmonar idiopática no Brasil

et al. 2019

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RESUMO Objetivo Ensaios clínicos mostraram que 150 mg de Nintedanibe duas vezes ao dia reduzem a progressão da doença em pacientes com Fibrose Pulmonar Idiopática (FPI), com um perfil de efeitos adversos que é controlável para a maioria dos pacientes. Antes da aprovação do Nintedanibe como tratamento para a FPI no Brasil, um Programa de Acesso Expandido (PEA) foi iniciado para fornecer acesso precoce ao tratamento e avaliar a segurança e a tolerância do Nintedanibe para este grupo de pacientes. Métodos Foram elegíveis para participar da PEA pacientes com diagnóstico de FPI nos últimos 5 anos, com capacidade vital forçada (CVF) ≥ 50% do previsto e capacidade de difusão dos pulmões para monóxido de carbono (DLco) 30%-79% do previsto. Os pacientes receberam Nintedanibe 150 mg, 2 vezes ao dia (bid). As avaliações de segurança incluíram eventos adversos que levaram à suspensão permanente do Nintedanibe e eventos adversos graves. Resultados O PEA envolveu 57 pacientes em 8 centros. A maioria dos pacientes era do sexo masculino (77,2%) e brancos (87,7%). No início do estudo, a média de idade foi de 70,7 (7,5) anos e a CVF foi de 70,7 (12,5%) do previsto. A média de exposição ao Nintedanibe foi de 14,4 (6,2) meses; a exposição máxima foi de 22,0 meses. Os eventos adversos frequentemente relatados pelo pesquisador como relacionados ao tratamento com Nintedanibe foram diarreia (45 pacientes, 78,9%) e náusea (25 pacientes, 43,9%). Os eventos adversos levaram à suspensão permanente do Nintedanibe em 16 pacientes (28,1%) que passaram por um evento adverso grave. Conclusões No PEA brasileiro, o Nintedanibe apresentou um perfil aceitável de segurança e tolerância em pacientes com FPI, condizendo com dados de ensaios clínicos.

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Pirfenidone and Nintedanib in idiopathic pulmonary fibrosis: Real-life experience in an Italian referral centre

Abstract: This study emphasizes that both antifibrotic drugs appeared to be a good therapeutic choice in terms of functional stabilization, also in older patients.

Cited by 50 publications

References 17 publications

Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena

Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena

Patient and site characteristics associated with pirfenidone and nintedanib use in the United States; an analysis of idiopathic pulmonary fibrosis patients enrolled in the Pulmonary Fibrosis Foundation Patient Registry

Segurança e tolerabilidade de Nintedanibe em pacientes com fibrose pulmonar idiopática no Brasil

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