Inhaled glutathione tolerability and efficacy in patients with cystic fibrosis

In CF patients, Glutathione (GSH), the first-line defence of lungs against oxidative stress, is severely reduced. A randomized, single blind controlled trial of inhaled GSH vs placebo (NCT01450267) is underway to evaluate the effect of GSH in a cohort of CF patients. 94 CF patients (48 F, median age 20.8 years) in regular follow up at the Regional Pediatric and Adult CF Center of Naples, were enrolled, 50 patients (23 F) were randomly assigned to the GSH group and 44 (25 F) to the placebo group. The inclusion criteria were: CF diagnosis by sweat test and/or two CF causing mutations, age of patients >6yrs, FEV1% >40% of the predicted value, negative culture for Burkholderia Cepacia. Spirometry was performed before, 10 and 60 minutes after GSH inhalation test (10 mg/Kg, maximum dosage 600mg/dose) in order to assess tolerability. Follow-up visits including spirometry took place one, three, six, nine months and after the end of treatments. No patients showed a decrease in FEV1% >15% after GSH inhalation. In the subgroup of CF patients (n=27) with an obstructive ventilatory defect (FEV1/FVC<88%) six months therapy with GSH determined a statistically significant increase in FEV1% (58.3 ± 13.2 vs 62.6 ± 15.1 p =0.048) compared to the group (n=20) treated with placebo (59.3 ± 14.9 vs 56.8 ± 18.4). Preliminary results suggest that inhaled GSH is well tolerated and determines an improvement in the respiratory function in CF patients.