Pirfenidone in Idiopathic Pulmonary Fibrosis: Expert Panel Discussion on the Management of Drug-Related Adverse Events

Abstract

Pirfenidone is currently the only approved therapy for idiopathic pulmonary fibrosis, following studies demonstrating that treatment reduces the decline in lung function and improves progression-free survival. Although generally well tolerated, a minority of patients discontinue therapy due to gastrointestinal and skin-related adverse events (AEs). This review summarizes recommendations based on existing guidelines, research evidence, and consensus opinions of expert authors, with the aim of providing practicing physicians with the specific clinical information needed to educate the patient and better manage pirfenidone-related AEs with continued pirfenidone treatment. The main recommendations to help prevent and/or mitigate gastrointestinal and skin-related AEs include taking pirfenidone during (or after) a meal, avoiding sun exposure, wearing protective clothing, and applying a broad-spectrum sunscreen with high ultraviolet (UV) A and UVB protection. These measures can help optimize AE management, which is key to maintaining patients on an optimal treatment dose.Electronic supplementary materialThe online version of this article (doi:10.1007/s12325-014-0112-1) contains supplementary material, which is available to authorized users.

Real-World Practice Patterns for Prevention and Management of Potential Adverse Events with Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis

“…In this survey, approximately half of the pulmonologists specifically advised their patients to take pirfenidone at mealtimes and provided specific recommendations on timing. A common and reportedly effective strategy used by these [6,9,12]. Pre-clinical and pharmacokinetic data have also indicated the potential effectiveness of administering pirfenidone with a substantial meal to reduce GI AEs.…”

“…For patients receiving pirfenidone, gastrointestinal (GI)-and skin-related adverse events (AEs) are the most common AEs reported [3][4][5]8]. These AEs can affect tolerability in some patients, particularly within the first 6 months of treatment [9]. In the phase III trials, the median time to the first GI AE (in 77.8% of patients), rash AE (35.8%), and photosensitivity AE (9.3%) was 14.0 (range, 5.0-40.0), 82.0 (range, 49.0-132.0), and 90.0 (range, 48.0-149.0) days, respectively [10].…”

“…One empirical method for preventing pirfenidone-related GI AEs is gradual dose titration upon initiation of pirfenidone [9]. Although an expert panel has recommended strategies for mitigating pirfenidone AEs and management strategies have been proposed on the basis of clinical trial data, real-world data on AE management in patients receiving pirfenidone therapy are limited [9,12].…”

“…Although an expert panel has recommended strategies for mitigating pirfenidone AEs and management strategies have been proposed on the basis of clinical trial data, real-world data on AE management in patients receiving pirfenidone therapy are limited [9,12]. Other realworld data analyses have focused on efficacy and tolerability of pirfenidone, physicians' expected use of IPF therapy based on disease severity in Europe and the United States, and treatment patterns and prescribing practices for antifibrotic therapy in patients with IPF in Europe [13][14][15].…”

A New Era in the Treatment of Scleroderma-associated Interstitial Lung Disease?

“…Indeed, pirfenidone can be associated with adverse events of the gastrointestinal (GI) system and the skin in patients with IPF, 2 organs very frequently involved in SSc-ILD. Sometimes these adverse events can lead to drug discontinuation 26 . If pirfenidone is efficient in SSc-ILD but worsens the GI manifestation of SSc, it could be a major problem for the future use of this drug in daily clinical practice.…”