Abstract
Purpose: Pirfenidone and nintedanib are two novel antifibrotic agents licensed for the treatment IPF. Prior to being approved for use in England for patients with FVC >50% and <80%, these were made available for all IPF patients under the Mild Patient Program (MPP) and Patient In Need Scheme (PIN). Prescribing of these medications is restricted to specialist centers. We sought to characterize the population of patients prescribed antifibrotics and determine the drug tolerability of these medications in the Northern hub of the Southwest of England regional ILD network.Methods: A retrospective analysis of all patients treated with antifibrotics between August 2012 and July 2017 was undertaken. Baseline characteristics including patient demographics and pulmonary physiology, in addition to drug tolerability and reasons for treatment cessation were collated. Data were compared using unpaired student’s t-test, Chi-squared, Mann–Whitney rank sum or ANOVA as appropriate. Logistic regression analysis evaluated clinical characteristics associated with discontinuation of pirfenidone therapy. P < 0.05 was considered statistically significant.Findings: A total of 164 patients, all with consensus diagnoses of IPF, were identified. Of these, 70.1% (115/164) received pirfenidone as their initial therapy. Baseline age, gender and pulmonary physiology did not differ significantly between groups. Drug discontinuation occurred most commonly due to adverse drug reactions events (ADRs) for both pirfenidone [40.0% (46/115)] and nintedanib [16.3% (8/49)]. Anorexia, rash and gastrointestinal disturbance were reported most commonly as the reason for cessation of pirfenidone; anorexia, nausea and weight loss for nintedanib. Duration of therapy prior to discontinuation because of ADRs did not differ significantly between medication groups but patients with a baseline FVC < 65% predicted, had a significantly shorter duration of pirfenidone prior to discontinuation due to ADRs, compared to those with a FVC 65–80% predicted. Multivariate logistic regression did not identify any independent baseline characteristics that predicted discontinuation of pirfenidone therapy prior to 52 weeks.Implications: Idiopathic pulmonary fibrosis (IPF) patients treated with nintedanib had comparable treatment emergent adverse event (TEAE) profiles in clinical practice to those reported in clinical trials. The TEAE profile of pirfenidone was higher than clinical trial data would suggest, although comparable to real-world datasets. Further work is required to explore the possible reasons underpinning this finding, including whether this is related to population co-morbidities or center threshold. No new safety concerns were identified.
Highlights
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease which leads to inexorable decline in lung function, culminating in respiratory failure and death with median survival of 2–5 years from diagnosis (Raghu et al, 2018).The Southwest region of England covers a large geographical area, occupying approximately 18% of England
Pending approval from the National Institute for Health and Clinical Excellence (NICE) institution in England, both medications were made available to patients by manufacturers under the remit of the mild patient program (MPP) for pirfenidone and the individual patient supply program (IPSP) and the patient in need (PIN) scheme for nintedanib
Two anti-fibrotic therapies are currently approved for the treatment of IPF based on the results of large scale phase III clinical trials (Noble et al, 2011; King et al, 2014; Richeldi et al, 2014)
Summary
The Southwest region of England covers a large geographical area, occupying approximately 18% of England. It has a population of approximately 4.6 million, accounting for 8.6% of the United Kingdom’s total population (Office for National Statistics, 2017). Two anti-fibrotic therapies are currently approved for the treatment of IPF. Pending approval from the National Institute for Health and Clinical Excellence (NICE) institution in England, both medications were made available to patients by manufacturers under the remit of the mild patient program (MPP) for pirfenidone and the individual patient supply program (IPSP) and the patient in need (PIN) scheme for nintedanib. Treatment should be stopped if there is evidence of disease progression (an absolute decline of 10% or more in predicted FVC within any 12 months period) (National Institute for Health and Care Excellence, 2013)
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