Aim: Pirfenidone has been recently approved for idiopathic pulmonary fibrosis-usual interstitial pneumonitis (IPF-UIP) in Japan & India. To the best of our knowledge, Indian data is not available. Reported side effects include photosensitivity, skin rash & gastrointestinal upset. We present our initial experience with tolerability of Pirfenidone in IPF-UIP. Methods: 15 patients (mean age 62 yrs, Male: 11) with clinical-radiological diagnosis of IPF-UIP were included. Baseline liver function, Spo2 & 2-D Echo were performed. Lung function tests & six minute walk tests were possible in 10 patients. Follow up oximetry & liver function tests were performed in all patients. Pirfenidone was started at 200 mg three times a day. Dose was increased to 400 mg three times a day after 2 to 4 weeks. Patients also received prednisolone 10 mg/day, n-acetyl cysteine 600 mg three times a day & & PPI. Results: At baseline mean Spo2 was 92.5%, mean FVC 1.42 Liters. Liver enzyme & bilirubin were normal. 8 patients had pulmonary hypertension on 2-D Echo. There was no significant increase in the liver enzymes at four weeks follow up. 4 patients were initiated on pirfenidone at diagnosis. Rest of patients were diagnosed with IPF-UIP at mean 27.6 months prior to initiating pirfenidone. Mean duration of follow up since starting pirfenidone was 52 days. 2 patients complained of nausea & 1 patient had loose motion. 2 patients skin itching, however, there was no discoloration. These patients were counseled about the adverse event, but preferred to continue the medication. Conclusion: At short term follow up, pirfenidone appears to be well tolerated in Indian patients with IPF-UIP.
Chaudhari et al. (Thu,) studied this question.