Pirfenidone Treatment in Individuals with Idiopathic Pulmonary Fibrosis: Impact of Timing of Treatment Initiation

Maher, Toby M.; Lancaster, Lisa; Jouneau, S.; Morrison, Lake; Lederer, David J.; Molina-Molina, María; Bendstrup, Elisabeth; Kirchgaessler, Klaus-Uwe; Gilberg, Frank; Axmann, Judit; Petzinger, Ute; Noble, Paul W.

doi:10.1513/annalsats.201810-720rl

Cited by 19 publications

(37 citation statements)

References 12 publications

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“…This may be due to providers and patients deferring anti-fibrotic initiation in patients with less severe disease. Such a strategy is not supported by evidence that shows anti-fibrotic use prevents irreversible lung function loss at all levels of disease severity [18]. There was decreasing use of anti-fibrotics with increased age; this may suggest that personalized decision-making results in deferral of anti-fibrotic use in patients of more advanced age for whom loss of future lung function might be of relatively lower concern when balanced against potential side effects and cost.…”

Section: Discussionmentioning

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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Section: Discussionmentioning

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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“…4 Patients with 'baseline' FVC < 50% of predicted in RECAP, which followed up patients who had completed ASCEND and CAPACITY, had a similar FVC trajectory compared with patients with better preserved FVC at baseline. 21 In INPULSIS-ON, 41 (6%) patients had an FVC < 50% at baseline. 5 It is notable that in all of these prior studies, patients had been followed up from the time of enrolment in the parent clinical trial.…”

Section: Discussionmentioning

confidence: 99%

“…In ASCEND, outcomes of patients who experienced a 10% relative decline in FVC during the trial were analysed during a subsequent defined follow‐up period, and appeared to have continued benefit from pirfenidone compared to placebo 4 . Patients with ‘baseline’ FVC < 50% of predicted in RECAP, which followed up patients who had completed ASCEND and CAPACITY, had a similar FVC trajectory compared with patients with better preserved FVC at baseline 21 . In INPULSIS‐ON, 41 (6%) patients had an FVC < 50% at baseline 5 .…”

Section: Discussionmentioning

confidence: 99%

Outcomes of patients with advanced idiopathic pulmonary fibrosis treated with nintedanib or pirfenidone in a real‐world multicentre cohort

et al. 2021

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Background and objective: Antifibrotic therapy with nintedanib or pirfenidone slows disease progression and reduces mortality in patients with idiopathic pulmonary fibrosis (IPF). However, patients with advanced IPF, as defined by forced vital capacity (FVC) < 50% and/or diffusion capacity for carbon monoxide (DLCO) < 30% of predicted, have not been included in randomized trials, and the outcomes of such patients who initiate treatment are not well understood. We determined lung function, disease progression and mortality outcomes following initiation of antifibrotic therapy in patients with advanced IPF at the time of treatment initiation compared to those with mild-moderate IPF. Methods: We included 502 patients enrolled in IPF registries from four Nordic countries. Linear mixed models were used to assess change in FVC and DLCO over time. Cox proportional hazards models were used to assess transplant-free survival and progression-and transplant-free survival. Results: Of 502 patients, 66 (13%) had advanced IPF. Annual change in FVC was À125 ml (95% CI À163, À87) among patients with mild-moderate IPF, and +28 ml (95% CI À96, +152) among those with advanced IPF. Advanced IPF at treatment initiation was associated with poorer transplant-free survival (hazard ratio [HR] 2.39 [95% CI 1.66, 3.43]) and progression-and transplant-free survival (HR 1.60 [95% CI 1.15, 2.23]). Conclusion: In a broadly representative IPF population, patients with advanced IPF at the initiation of antifibrotic therapy did not have greater lung function decline over time compared with those with mild-moderate IPF, but had substantially higher mortality. Prospective studies are needed to determine the effect of antifibrotic therapy in patients with advanced IPF.

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“…Some health authorities have consequently modified eligibility, forgoing or extending deadlines for completion of PFTs and allowing alternative methods of diagnostic confirmation. This approach is supported by the consistent benefits of antifibrotic therapy across severities of IPF and in patients with non-IPF fibrotic ILD, [28][29][30][31][32][33][34][35] suggesting that temporary loosening of previous criteria may be preferable to restricting medication access.…”

Section: Antifibrotic Medicationsmentioning

confidence: 97%

Practical Considerations for the Diagnosis and Treatment of Fibrotic Interstitial Lung Disease During the Coronavirus Disease 2019 Pandemic

Wong

Fidler

Marcoux

et al. 2020

Chest

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The coronavirus disease 2019 (COVID-19) pandemic, caused by severe acute respiratory syndrome coronavirus 2, has affected virtually all aspects of patient care. Health-care systems around the world are trying simultaneously to treat patients with COVID-19, prepare for its longterm impacts, and treat patients with other acute and chronic diseases. There are multiple ways that the COVID-19 pandemic will directly affect patients with fibrotic interstitial lung disease (ILD), particularly given their common risk factors for poor outcomes. Major issues for patients with ILD will include restricted access to key components of the diagnostic process, new uncertainties in the use of common ILD pharmacotherapies, limited ability to monitor both disease severity and the presence of medication adverse effects, and significantly curtailed research activities. The purpose of this review is to summarize how COVID-19 has impacted key components of the diagnosis and management of fibrotic ILD as well as to provide strategies to mitigate these challenges. We further review major obstacles for researchers and identify priority areas for future ILD research related to COVID-19. Our goals are to provide practical considerations to support the care of patients with ILD during the COVID-19 pandemic and to provide a road map for clinicians caring for these patients during future infectious disease outbreaks.

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Pirfenidone Treatment in Individuals with Idiopathic Pulmonary Fibrosis: Impact of Timing of Treatment Initiation

Cited by 19 publications

References 12 publications

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

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

Outcomes of patients with advanced idiopathic pulmonary fibrosis treated with nintedanib or pirfenidone in a real‐world multicentre cohort

Practical Considerations for the Diagnosis and Treatment of Fibrotic Interstitial Lung Disease During the Coronavirus Disease 2019 Pandemic

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