Drug Treatment of Idiopathic Pulmonary Fibrosis

Canestaro, William J.; Forrester, Sara; Raghu, Ganesh; Ho, Lawrence; Devine, Beth

doi:10.1016/j.chest.2015.11.013

Cited by 153 publications

(72 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The clinical inputs of the economic analysis were based on an NMA conducted after a systematic review of the literature. The NMA results were similar with three other studies [42, 46, 47]. The OS parametric extrapolation estimates used in the base-case analysis were validated with external observational data [5, 19].…”

Section: Discussionsupporting

confidence: 82%

A Cost-Effectiveness Analysis of Nintedanib in Idiopathic Pulmonary Fibrosis in the UK

Rinciog¹,

Watkins

Chang³

et al. 2016

PharmacoEconomics

View full text Add to dashboard Cite

BackgroundInternational guidelines recommend nintedanib (OFEV®) as an option for the treatment of idiopathic pulmonary fibrosis (IPF).ObjectiveThe objective of this study was to assess the cost effectiveness of nintedanib versus pirfenidone, N-acetylcysteine and best supportive care (BSC) for the treatment of IPF from a UK payer’s perspective.MethodsA Markov model was designed to capture the changes in the condition of adults with IPF. Efficacy outcomes included mortality, lung function decline and acute exacerbations. Treatment safety (serious adverse events) and tolerability (overall discontinuation) were also considered. The baseline risk of these events was derived from patient-level data from the placebo arms of nintedanib clinical trials (TOMORROW, INPULSIS-1, INPULSIS-2). A network meta-analysis (NMA) was conducted to estimate the relative effectiveness of the comparator treatments. Quality of life and healthcare resource use data from the clinical trials were also incorporated in the economic model.ResultsNintedanib showed statistically significant differences against placebo on acute exacerbation events avoided and lung function decline. In the cost-effectiveness analysis, the results were split between two treatments with relative low costs and modest effectiveness (BSC and N-acetylcysteine) and two that showed improved effectiveness (lung function) and higher costs (nintedanib and pirfenidone). All comparators were assumed to have similar projected survival and the difference in quality-adjusted life-years (QALYs) was driven by the acute exacerbations and lung function estimates. In the base-case deterministic pairwise comparison with pirfenidone, nintedanib was found to have fewer acute exacerbations and resulted in less costs and more QALYs gained.ConclusionsCompared with BSC (placebo), nintedanib and pirfenidone were the only treatments to show statistical significance in the efficacy parameters. We found substantial uncertainty in the overall cost-effectiveness results between nintedanib and pirfenidone. N-Acetylcysteine was largely similar to BSC but with a worse survival profile.INPULSIS-1 and INPULSIS-2 ClinicalTrials.gov numbers, NCT01335464 and NCT01335477Electronic supplementary materialThe online version of this article (doi:10.1007/s40273-016-0480-2) contains supplementary material, which is available to authorized users.

show abstract

Section: Discussionsupporting

confidence: 82%

A Cost-Effectiveness Analysis of Nintedanib in Idiopathic Pulmonary Fibrosis in the UK

Rinciog¹,

Watkins

Chang³

et al. 2016

PharmacoEconomics

View full text Add to dashboard Cite

show abstract

“…Median survival is 3–5-years, shorter than for many malignancies [4]. The antifibrotic drugs, pirfenidone and nintedanib, slow lung function decline but have not been convincingly shown to improve survival or quality of life (QoL) [5, 6]. Beside prolonging survival, major aims for IPF therapy include improving symptoms and QoL domains like physical functioning, social participation and emotional well-being [7].…”

Section: Introductionmentioning

confidence: 99%

Health related quality of life in patients with idiopathic pulmonary fibrosis in clinical practice: insights-IPF registry

et al. 2017

View full text Add to dashboard Cite

BackgroundThe INSIGHTS-IPF registry provides one of the largest data sets of clinical data and self-reported patient related outcomes including health related quality of life (QoL) on patients with idiopathic pulmonary fibrosis (IPF). We aimed to describe associations of various QoL instruments between each other and with patient characteristics at baseline.MethodsSix hundred twenty-three IPF patients with available QoL data (St George’s Respiratory Questionnaire SGRQ, UCSD Shortness-of-Breath Questionnaire SoB, EuroQol visual analogue scale and index EQ-5D, Well-being Index WHO-5) were analysed. Mean age was 69.6 ± 8.7 years, 77% were males, mean disease duration 2.0 ± 3.3 years, FVC pred was 67.5 ± 17.8%, DLCO pred 35.6 ± 17%.ResultsMean points were SGRQ total 48.3, UCSD SoB 47.8, EQ-5D VAS 66.8, and WHO-5 13.9. These instruments had a high or very high correlation (exception WHO-5 to EQ-5D VAS with moderate correlation). On bivariate analysis, QoL by SGRQ total was statistically significantly associated with clinical symptoms (NYHA; p < 0.001), number of comorbidities (p < 0.05), hospitalisation rate (p < 0.01) and disease severity (as measured by GAP score, CPI, FVC and 6-min walk test; p < 0.05 each). Multivariate analyses showed a significant association between QoL (by SGRQ total) and IPF duration, FVC, age, NYHA class and indication for long-term oxygen treatment.ConclusionsOverall, IPF patients under real-life conditions have lower QoL compared to those in clinical studies. There is a meaningful relationship between QoL and various patient characteristics.Trial registrationThe INSIGHTS-IPF registry is registered at Clinicaltrials.gov (NCT01695408).Electronic supplementary materialThe online version of this article (doi:10.1186/s12931-017-0621-y) contains supplementary material, which is available to authorized users.

show abstract

“…It remains to be determined whether pirfenidone, an antifibrotic agent that inhibits transforming growth factor-beta-stimulated collagen synthesis, extracellular matrix deposition, and fibroblast proliferation, or nintedanib, a receptor blocker for multiple tyrosine kinases that mediate elaboration of fibrogenic growth factors, will be efficacious in these patients. ese agents appear to slow disease progression [25,26] and decrease mortality rates [27,28] in patients with IPF.…”

Section: Discussionmentioning

confidence: 99%

Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease

Clifford

Renaud

et al. 2019

Case Reports in Rheumatology

View full text Add to dashboard Cite

Background. Microscopic polyangiitis (MPA) can manifest with atypical features such as pulmonary fibrosis and chronic obstructive pulmonary disease (COPD), which are atypical and unusual features of small vessel vasculitis. Case Presentation. This paper presents two patients with microscopic polyangiitis and respiratory symptoms attributable to atypical pulmonary manifestations. Pulmonary fibrosis was present in both cases, with COPD also present in one patient. Management involved methylprednisone, prednisone, and cyclophosphamide. The second patient also received azathioprine. Both patients responded well to immunosuppressive treatment; however, pulmonary fibrosis and COPD were refractory to immunosuppression. Conclusion. Pulmonary manifestations including pulmonary fibrosis, emphysema, and bronchiectasis are observed in MPA. Evaluation of MPA in unexplained cases should be performed to avoid delays in diagnosis and management. Patients who present with MPA with pulmonary manifestations may respond to treatment, but their pulmonary features demonstrate a refractory nature to such management.

show abstract

Drug Treatment of Idiopathic Pulmonary Fibrosis

Cited by 153 publications

References 27 publications

A Cost-Effectiveness Analysis of Nintedanib in Idiopathic Pulmonary Fibrosis in the UK

A Cost-Effectiveness Analysis of Nintedanib in Idiopathic Pulmonary Fibrosis in the UK

Health related quality of life in patients with idiopathic pulmonary fibrosis in clinical practice: insights-IPF registry

Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease

Contact Info

Product

Resources

About