Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature

Nalysnyk, Luba; Cid‐Ruzafa, Javier; Rotella, Philip; Eßer, Dirk

doi:10.1183/09059180.00002512

Cited by 458 publications

(361 citation statements)

References 32 publications

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“…I dati sull'incidenza e la prevalenza della IPF sono scarsi e le stime effettuate nei diversi Stati differiscono anche a causa delle diverse definizioni e metodologie applicate [1], rendendo il confronto molto difficile. Ne deriva che la reale prevalenza ed incidenza dell'IPF non può essere stabilita con certezza [2]. Alcune recenti revisioni della letteratura [3,4] hanno evidenziato i diversi punti di forza e debolezza dei vari studi, del loro disegno e le principali problematiche che possono emergere, quando vengono effettuati dei confronti tra i differenti studi, rendendo pertanto difficile o impossibile giungere ad incidenze e prevalenze globali e stimare la mortalità attraverso metanalisi.…”

Section: Epidemiologia Della Ipfunclassified

[Nintedanib for the treatment of idiopathic pulmonary fibrosis in Italy]

Belisari¹,

Bettoni

Cortesi

et al. 2017

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To date, there are few therapeutic answers for Idiopathic pulmonary fibrosis (IPF) and only two pharmacological treatments have a marketing authorization for this disease. Recently nintedanib (Ofev®) has been authorized as a new therapeutic option and its economic profile has been evaluated by international Health Technology Assessment (HTA) bodies. IPF has important implications for everyday life of patients and their carers, negatively influencing their quality of life and bringing heavy economic burden to the NHS and to the entire society. It is, therefore important to consider these aspects for the Italian environment and to perform a pharmacoeconomic evaluation to define the efficiency of nintedanib in IPF by means of a Cost-Utility Analysis (CUA). As IPF is a chronic and progressive disease, a lifetime Markov model has been therefore developed with health states describing the patient’s condition as a combination of lung function and exacerbation history. The cohort entered in the model at different Forced Vital Capacity (FVC%) predicted health states, without exacerbation. The Clinical data used to perform this CUA were derived from clinical trials and the relative efficacy of nintedanib versus the comparator (pirfenidone) was then obtained from a Network Meta-Analysis (NMA) combining data reported in each primary study (INPULSIS 1-2 and TOMORROW trials for nintedanib, and CAPACITY and ASCEND trials for pirfenidone, respectively). At base-case, treatment with nintedanib resulted in a slightly lower estimated total cost vs pirfenidone, better safety profile and lower risk of acute exacerbations with an advantage in Quality Adjusted Life years (QALYs) gained. These results were confirmed by the sensitivity analysis. Although nintedanib appears to be a valuable option for the NHS to treat IPF patients, future data evidence, as long-term or real-life data, will help to confirm these results.[In Italian]

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Section: Epidemiologia Della Ipfunclassified

[Nintedanib for the treatment of idiopathic pulmonary fibrosis in Italy]

Belisari¹,

Bettoni

Cortesi

et al. 2017

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“…(Frankel & Schwarz, 2009;Nalysnyk et al, 2012;Raghu et al, 2015). Em todo o mundo, aproximadamente 5 milhões de pessoas são acometidas com FPI, com prevalência mais elevada entre homens do que entre mulheres (20 por 100.000 e 13 por 100.000, respectivamente) (Meltzer & Noble, 2008), com diagnóstico, em média, aos 66 anos (American Thoracic Society (ATS) & European Respiratory Society (ERS), 2000; du Bois, 2012).…”

Section: Introductionunclassified

“…Assim, a FPI constitui uma doença crônica, progressiva, com um curso inexorável e devastador, que apresenta um desfecho fatal, com impacto negativo na qualidade de vida dos pacientes e de suas famílias e elevada carga econômica imposta ao sistema de saúde (Frankel & Schwarz, 2009;Nalysnyk et al, 2012;Raghu et al, 2015).…”

Section: Introductionunclassified

Análise de custo-efetividade de pirfenidona em comparação a nintedanibe no tratamento de fibrose pulmonar idiopática na perspectiva do sistema suplementar de saúde brasileiro

Ho¹,

Ltd.²,

Rufino³

et al. 2017

JBES

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Objetivo: O objetivo do estudo foi avaliar o custo-efetividade de pirfenidona em comparação ao nintedanibe no tratamento de pacientes com fibrose pulmonar idiopática (FPI) na perspectiva do sistema suplementar de saúde. Métodos: O modelo foi realizado considerando um horizonte de tempo lifetime. O principal desfecho da análise foram os anos de vida ganhos. Os custos de aquisição de medicamentos foram obtidos por meio das listas oficiais de preço, considerando o ICMS 18%. Os demais custos relacionados ao manejo da doença, transplante de pulmão e custo de final de vida foram calculados por um microcusteio baseado em opinião de especialistas e listas de preços de procedimentos. Os dados de eficácia foram extraídos dos estudos CAPACITY 1, 2 e ASCEND, e extrapolados por meio de uma distribuição paramétrica Weibull. Os dados referentes ao nintedanibe foram extrapolados por uma comparação indireta. Resultados: Os custos totais de pirfenidona e de nintedanibe foram R$ 319.689 e R$ 522.887, respectivamente. Os anos de vida salvos resultantes foram 6,536 para pirfenidona e 5,726 para nintedanibe, resultando em um valor incremental de 0,810. Conclusão: Dessa maneira, a partir dos valores incrementais de custos e efetividade, a pirfenidona demonstrou ser uma opção terapêutica dominante quando comparada ao nintedanibe.

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“…Hence, the prevalence and annual incidence of IPF in the USA varies from 14 to 27.9 cases per 100 000 population and 6. 8-8.8 per 100 000 population, respectively, using narrow case definitions, and from 42.7 to 63 cases per 100,000 population and 16.3-17.4 per 100 000 population, respectively, using broad case definitions [3]. In Europe, the prevalence and annual incidence ranges from 1.25 to 23.4 cases per 100 000 population and 0.…”

Section: Introductionmentioning

confidence: 99%

“…In Europe, the prevalence and annual incidence ranges from 1.25 to 23.4 cases per 100 000 population and 0. 22-7.4 per 100 000 population, respectively [3].…”

Section: Introductionmentioning

confidence: 99%

Pharmacological treatment of idiopathic pulmonary fibrosis: from the past to the future

2013

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During the past decade important progress has been made regarding the pathogenesis of idiopathic pulmonary fibrosis (IPF), which is the most devastating form of idiopathic interstitial pneumonia with a median survival of 3 years. The knowledge gained has been used to design multicentre, randomised, placebo-controlled trials in order to investigate agents with different mechanisms of action. Encouraging results have led to licensing of the first IPF-specific drug, pirfenidone. However, the road to successful treatment is still long. The main aim for the future should be the careful design of clinical trials, by choosing the most clinically meaningful end-point and keeping in mind that combination of various agents may be more effective. This approach has been used in the treatment of lung cancer with which IPF presents many similarities. @ERSpublications Progress toward the treatment of IPF and aims for future clinical trials

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Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature

Cited by 458 publications

References 32 publications

[Nintedanib for the treatment of idiopathic pulmonary fibrosis in Italy]

[Nintedanib for the treatment of idiopathic pulmonary fibrosis in Italy]

Análise de custo-efetividade de pirfenidona em comparação a nintedanibe no tratamento de fibrose pulmonar idiopática na perspectiva do sistema suplementar de saúde brasileiro

Pharmacological treatment of idiopathic pulmonary fibrosis: from the past to the future

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