HealthDay News – Two treatments — pirfenidone and nintedanib — but not acetylcysteine, correlated with improvements in forced vital capacity (FVC) among patients with idiopathic pulmonary fibrosis, according to three research findings that were published in the New England Journal of Medicine.

“Idiopathic pulmonary fibrosis is a chronic, progressive, and fatal lung disease that is characterized by irreversible loss of lung function,” Talmadge E. King Jr., MD, and colleagues wrote. “Although periods of transient clinical stability may be observed, continued progression of the disease is inevitable.The prognosis is poor, with a 5-year survival rate that is similar to the rates for several cancers.”

After assigning 555 randomized patients to receive oral pirfenidone or a placebo for 52 weeks, the inspectors observed a 47.9% relative reduction in the proportion of patients with an absolute decline of 10% or more in the percentage of predicted FVC or patients who died in the pirfenidone group (P<0.001).

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A second, separate phase 3 study assessed the efficacy and safety of the investigational drug nintedanib. Luca Richeldi, MD, PhD, and colleagues conducted two replicate 52-week trials (INPULSIS-1 and INPULSIS-2) to assess outcomes among patients assigned to 150 mg of nintedanib twice daily compared with placebo. They found the annual rate of change in FVC was significantly reduced for nintedanib compared with placebo (difference, 125.3 mL in INPULS-1; 93.7 mL per year in INPULSE-2; P<0.001 for both).

“In patients with idiopathic pulmonary fibrosis, nintedanib reduced the decline in FVC, which is consistent with a slowing of disease progression,” wrote the researchers.

Despite the observed effectiveness of pirfenidone and nintedanib in trials, patients with idiopathic pulmonary fibrosis with mild-to-moderate impairment in pulmonary function experienced no significant benefit from acetylcysteine, according to a third study by Fernando J. Martinez, MD, and colleagues. Their results were based on an initial double-blind, placebo-controlled trial.

“Treatment with acetylcysteine did not help preserve the FVC in patients with idiopathic pulmonary fibrosis,” wrote the researchers.


  1. King, T et al. New England Journal of Medicine. 2014; doi: 10.1056/NEJMoa1402582
  2. Richeldi, L et al. New England Journal of Medicine. 2014; doi: 10.1056/NEJMoa1402584
  3. Martinez, F et al. New England Journal of Medicine. 2014; doi: 10.1056/NEJMoa1401739


The King study was supported by InterMune. Several authors from the Richeldi study disclosed financial ties to pharmaceutical companies, including Boehringer Ingelheim, which supported the study. Zambon provided the acetylcysteine and matching placebo used in the Martinez study.