Idiopathic pulmonary fibrosis (IPF) lacks effective treatment. Pirfenidone has been used to treat IPF patients. N-acetylcysteine (NAC) exerts antioxidant and antifibrotic effects on IPF cases.This study is a double-blind, modified placebo-controlled, randomized phase II trial of pirfenidone in Chinese IPF patients. We randomly assigned the enrolled Chinese IPF patients with mild to moderate impairment of pulmonary function to receive either oral pirfenidone (1800 mg per day) and NAC (1800 mg per day) or placebo and NAC (1800 mg per day) for 48 weeks. The primary endpoints were the changes in forced vital capacity (FVC) and walking distance and the lowest SPO2 during the 6-minute walk test (6MWT) at week 48. The key secondary endpoint was the progression-free survival time. This study is registered in ClinicalTrials.gov as number NCT01504334.Eighty-six patients were screened, and 76 cases were enrolled (pirfenidone + NAC: 38; placebo + NAC: 38). The effect of pirfenidone treatment was significant at the 24th week, but this effect did not persist to the 48th week. At the 24th week, the mean decline in both FVC and ΔSPO2 (%) during the 6MWT in the pirfenidone group was lower than that in the control group (−0.08 ± 0.20 L vs −0.22 ± 0.29 L, P = 0.02 and −3.44% ± 4.51% vs −6.29% ± 6.06%, P = 0.03, respectively). However, there was no significant difference between these 2 groups at the 48th week (−0.15 ± 0.25 L vs −0.25 ± 0.28 L, P = 0.11 and −4.25% ± 7.27% vs −5.31% ± 5.49%, P = 0.51, respectively). The pirfenidone treatment group did not achieve the maximal distance difference on the 6MWT at either the 24th or the 48th week. But pirfenidone treatment prolonged the progression-free survival time in the IPF patients (hazard ratio = 1.88, 95% confidence interval: 1.092–3.242, P = 0.02). In the pirfenidone group, the adverse event (AE) rate (52.63%) was higher than that in the control group (26.3%, P = 0.03). Rash was more common in the pirfenidone group (39.5% vs 13.2%, P = 0.02).Compared with placebo combined with high-dose NAC, pirfenidone combined with high-dose NAC prolonged the progression-free survival of Chinese IPF patients with mild to moderate impairment of pulmonary function. (ClinicalTrials.gov number, NCT01504334).

Study Objectives: Despite the clinical and prognostic significance of obstructive sleep apnea (OSA) in chronic respiratory diseases (CRDs), there have been few studies about the possible predictors of OSA and the effect of OSA on quality of life in patients with CRDs. The objectives were to identify physiological and clinical parameters that predict the occurrence and severity of OSA and to investigate the effect of OSA on quality of life in patients with CRDs. Methods: Seventy-three patients with chronic obstructive pulmonary disease (COPD) and 77 patients with fibrotic interstitial lung disease (ILD) underwent overnight polysomnography (PSG) and pulmonary function testing and completed clinical questionnaires. The oximetry tracing was interpreted blindly with respect to the PSG results. Results: The prevalence of OSA was 44% and 62% in COPD and ILD, respectively. The COPD assessment test item scores related to sleep quality and daily vitality were worse among patients with OSA than among patients without OSA. The STOP-BANG questionnaire (cutoff point ≥ 3) and oxygen desaturation index from the oximetry recording (oxygen desaturation index (ODI) were associated with OSA in CRDs. The STOP-BANG questionnaire with a cutoff point ≥ 3 or 6 had the highest sensitivity and specificity, respectively, in detecting OSA in CRDs. ODI had the best accuracy in identifying OSA and was independently associated with the apnea-hypopnea index in CRDs. Conclusions: We found OSA to be common and associated with worse sleep quality and less daily vitality in patients with advanced CRDs. The STOP-BANG questionnaire with different cutoff points may help rule in or rule out OSA. Overnight oximetry can be used as a screening tool for OSA and can assist the clinical evaluation of OSA in patients with CRDs.