Antifibrotic Drug Use in Patients with Idiopathic Pulmonary Fibrosis. Data from the IPF-PRO Registry

Abstract

Rationale: Two antifibrotic medications, nintedanib and pirfenidone, have been approved for the treatment of idiopathic pulmonary fibrosis (IPF) in the United States. Few data have been published on the use of these medications in clinical practice. Objectives: To investigate patterns of use of antifibrotic medications in the United States. Methods: The Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry, a multicenter U.S. registry, has enrolled patients with IPF that was diagnosed or confirmed at the enrolling center in the past 6 months. Data from patients enrolled from June 5, 2014, to March 4, 2018, were used to determine antifibrotic medication use (“treatment”) in the enrollment window and in a follow-up window approximately 6 months later. Associations between patient characteristics and treatment status were tested using logistic regression. Results: Overall, 551 of 782 eligible patients (70.5%) were treated in the enrollment window. Younger age, lower forced vital capacity percentage predicted, oxygen use with activity, worse self-rated health (based on the Short Form 12 or St. George’s Respiratory Questionnaire score), referral to the enrolling center by a pulmonologist, use of a lung biopsy in diagnosis, and carrying a diagnosis of IPF to the enrolling center were associated with being treated. Among 534 patients treated at enrollment who had follow-up data, 94.0% remained treated in follow-up. Better self-rated health (based on the Short Form 12 mental component score or EuroQoL score) and not using oxygen with activity at enrollment were associated with continuing treatment in follow-up. Among 172 patients who were untreated at enrollment and had follow-up data, 29.7% started treatment in follow-up. Lower diffusing capacity of the lung for carbon monoxide percentage predicted, a family history of interstitial lung disease, a history of sleep apnea, and a definite diagnosis of IPF at enrollment were associated with starting treatment in follow-up. Conclusions: The majority of patients in the IPF-PRO Registry were receiving an approved medication for IPF at enrollment. Treatment at enrollment was associated with greater disease severity, more compromised quality of life, and the use of oxygen with activity. Clinical trial registered with ClinicalTrials.gov (NCT01915511).

Reply: Adoption of Antifibrotic Medications: A Closer Look at the Data

“…As discussed in the manuscript, we are in agreement with Dr. White and colleagues that referral to a pulmonary physician (and ideally a multi-disciplinary group including chest radiologists, pulmonary pathologists, rheumatologists, and thoracic surgeons) is critical for the management of a complex disease like IPF and that the observed lack of referrals in our study may have contributed to the lower-than-expected adoption rate. This finding likely also explains the significant difference in adoption found in our analysis and those of various United States IPF registries where involvement of a pulmonary consultant and multi-disciplinary group is an essential component of care (2,3).…”

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

“…20 In practice, however, patients with advanced IPF are commonly treated with nintedanib or pirfenidone, with as many as one in five treated patients having FVC and/or DLCO values below the thresholds for inclusion in trials. 3 Existing insights into the outcomes of such patients have come largely from secondary analyses of clinical trials or from open-label post-authorization studies. 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.…”

Monitoring and management of fibrosing interstitial lung diseases: a narrative review for practicing clinicians

“…Among 782 patients in a large US registry, most patients taking an antifibrotic therapy at enrollment were still taking the same therapy approximately 6 months later. 69 …”