Source: International Congress 2014 – ILDs 6
Disease area: Interstitial lung diseases

Abstract

Introduction: Pirfenidone (Esbriet^®; PFD) is the only drug indicated for the treatment of mild to moderate idiopathic pulmonary fibrosis (IPF). Due to the complex pathophysiology of IPF involving multiple pathways, combination therapy seems to be the future. A candidate drug is N-acetylcysteine (NAC), a mucolytic and antioxidant agent extensively studied in respiratory disorders, including IPF. The design of an on-going study of NAC and PFD in IPF is presented.Aims and objectives: To evaluate the safety and tolerability of NAC in combination with PFD when PFD is prescribed as the standard of care in IPF patients.Methods: In this Phase 2, double-blind, placebo-controlled trial, patients are randomized (1:1, ~125 per group) to receive either NAC (1800 mg/day) or placebo in combination with PFD (2403 mg/day orally). Eligible patients (40–80 years) have IPF diagnosed according to current guidelines and baseline pulmonary function tests in the range 50% £FVC £90%, [FEV₁/FVC] ³0.75, and 30% £DL_CO £90% (assessed by central review). Important exclusion criteria are history of significant environmental exposure known to cause pulmonary fibrosis and a known cause of interstitial lung disease. The study consists of 3 periods: washout and screening (days -42 to -28); treatment (24 weeks); follow-up (28 days after treatment completion).Results: Since June 2013 approximately 100 patients have been enrolled. Baseline data will be presented. They show that the patient population is comparable to those of previous PFD studies.Conclusions: This is the first safety and tolerability study of NAC in combination with PFD for the treatment of IPF.

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J. Behr, E. Bendstrup, B. Crestani, A. Günther, H. Olschewski, M. Skoeld, A. Wells, W. Wuyts, H. Tang, J. Beck, C. Albera (Munich, Giessen, Germany; Aarhus C, Denmark; Paris, France; Graz, Austria; Stockholm, Sweden; London, United Kingdom; Leuven, Belgium; Brisbane, United States Of America; Muttenz, Switzerland; Orbassano (Turin), Italy). A Phase 2, double-blind, placebo-controlled study of N–acetylcysteine in combination with pirfenidone in patients with idiopathic pulmonary fibrosis. Eur Respir J 2014; 44: Suppl. 58, 3786

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