title: 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.
authors.affiliation: Department of Pneumology/Allergology, Ruhrlandklinik, University Hospital, University Duisburg-Essen, Essen, Germany, ulrich.costabel@ruhrlandklinik.uk-essen.de.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Costabel
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Bendstrup
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.forename: Pieter
authors.initials: P
authors.lastname: Dewint
authors.forename: Jim J J
authors.initials: JJ
authors.lastname: Egan
authors.forename: James
authors.initials: J
authors.lastname: Ferguson
authors.forename: Richard
authors.initials: R
authors.lastname: Groves
authors.forename: Per M
authors.initials: PM
authors.lastname: Hellström
authors.forename: Michael
authors.initials: M
authors.lastname: Kreuter
authors.forename: Toby M
authors.initials: TM
authors.lastname: Maher
authors.forename: Maria
authors.initials: M
authors.lastname: Molina-Molina
authors.forename: Klas
authors.initials: K
authors.lastname: Nordlind
authors.forename: Alexandre
authors.initials: A
authors.lastname: Sarafidis
authors.forename: Carlo
authors.initials: C
authors.lastname: Vancheri
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage / Q000009:adverse effects; D019468:Disease Management; D018572:Disease-Free Survival; D064420:Drug-Related Side Effects and Adverse Reactions / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000188:drug therapy; D011728:Pyridones / Q000008:administration & dosage / Q000009:adverse effects; D012129:Respiratory Function Tests / Q000379:methods; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; D014409:Tumor Necrosis Factor-alpha; C093844:pirfenidone