title: Effectiveness of combined therapy with pirfenidone and inhaled N-acetylcysteine for advanced idiopathic pulmonary fibrosis: a case-control study.
abstract: OBJECTIVE
  Treatment with pirfenidone may slow the decline in vital capacity and increase progression-free survival (PFS) in idiopathic pulmonary fibrosis (IPF). The effects of combination therapy with inhaled N-acetylcysteine (NAC) and pirfenidone are unclear. We assessed the effects of this combination therapy in patients with advanced IPF.
  
  
  METHODS
  Patients with a diagnosis of advanced IPF (Japanese Respiratory Society stage III/IV IPF) and a relative decline in forced vital capacity (FVC) of ≥ 10% within the previous 6 (± 2) months were enrolled. Outcomes were evaluated in a 12-month follow-up pulmonary function test. Treatment was considered ineffective if the decline in FVC was ≥ 10% and effective if the decline was <10%. We compared clinical characteristics, effectiveness and PFS between patients receiving inhaled NAC plus pirfenidone (n = 24) and those receiving pirfenidone alone (control; n = 10).
  
  
  RESULTS
  Data from 34 IPF patients (age range, 59-82 years) were analysed. At the 12-month follow-up examination, treatment was deemed effective in 8 of 17 (47%) patients receiving NAC plus pirfenidone and in 2 of 10 (20%) receiving pirfenidone alone. The annual rate of change in FVC was -610 mL in the NAC plus pirfenidone group and -1320 mL in the pirfenidone group (P < 0.01). PFS was longer (304 days) in the NAC plus pirfenidone group than in the pirfenidone group (168 days; P = 0.016).
  
  
  CONCLUSIONS
  Combination treatment with inhaled NAC and oral pirfenidone reduced the rate of annual FVC decline and improved PFS in patients with advanced IPF.
authors: Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan.
authors: Susumu
authors: S
authors: Sakamoto
authors: Yoko
authors: Y
authors: Muramatsu
authors: Keita
authors: K
authors: Satoh
authors: Fumiaki
authors: F
authors: Ishida
authors: Naoshi
authors: N
authors: Kikuchi
authors: Go
authors: G
authors: Sano
authors: Keishi
authors: K
authors: Sugino
authors: Kazutoshi
authors: K
authors: Isobe
authors: Yujiro
authors: Y
authors: Takai
authors: Sakae
authors: S
authors: Homma
mesh_terms: D000111:Acetylcysteine / Q000008:administration & dosage*; D000280:Administration, Inhalation; D000368:Aged; D000369:Aged, 80 and over; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage; D018572:Disease-Free Survival; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D005260:Female; D016166:Free Radical Scavengers / Q000008:administration & dosage; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011728:Pyridones / Q000008:administration & dosage*; D012189:Retrospective Studies; D016896:Treatment Outcome; D014797:Vital Capacity / Q000187:drug effects
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D016166:Free Radical Scavengers; D011728:Pyridones; C093844:pirfenidone; D000111:Acetylcysteine
keywords: N-acetylcysteine; advanced idiopathic pulmonary fibrosis; combination therapy; pirfenidone