نبذة مختصرة : Background: Idiopathic pulmonary fibrosis (IPF) is a form of progressive fibrosing interstitial lung disease with a poor prognosis. Pirfenidone is an oral antifibrotic agent used in the treatment of IPF, and it reduces the rate of decline of lung function. However, the tolerable dose and adverse effect profile appear to be different in the Indian population. Methodology: This prospective observational study was conducted in the Department of Pulmonary Medicine at a tertiary care center in India between January 2015 and June 2016. All adult patients diagnosed as IPF based on multidisciplinary discussion were enrolled. Subjects with active respiratory tract infection, other end-organ failures, and drugs interacting with Pirfenidone were excluded. Data collection was done with a predesigned questionnaire at baseline. Subsequently, they were followed up telephonically every week and in-person review at 3 and 6 months. Results: The cohort has 30 patients with a mean age of 60 years ± 4.5 (standard deviation 8.9). 80% did not tolerate the recommended total dose (40 mg/kg/day or 2400 mg/kg) and the median tolerated dose was 1800 mg/day (25–30 mg/kg). 75% reported adverse events, the most common being anorexia, and gastroesophageal reflux disease in gastrointestinal (GI) reaction, followed by a rash in dermatological reactions. All these adverse effects were mild to moderate and managed with supportive care or dose reduction. None of them were severe, causing cessation of therapy. Conclusion: Pirfenidone has a lower tolerable dose and predominantly GI adverse effects in our population, and if addressed early, it can prevent cessation of treatment and better quality of life for patients.
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