EFFICIENCY EVALUATION OF PIRFENIDONE IN EGYPTIAN IDIOPATHIC PULMONARY FIBROSIS PATIENTS

Background:. Idiopathic pulmonary fibrosis (IPF) is a complicated disease in which histopathological abnormalities in the form of, proliferation of mesenchymal cell, fibrosis, overproduction and disorganization of collagen deposition, extra cellular matrix changes with distortion of pulmonary structure and the appearance of subpleural cysts , with accumulation of both myofibroblast and fibroblast. Pirfenidone has been available in Japan since 2008.In 2011, the approval by the European Medicine Agency (EMA) was indicated by the efficacy of pirfenidone in decreasing lung function deterioration in IPF patients. Aim of the Study: The aim of the study is to study the efficiency and safety of Pirfenidone in a group of IPF Patients. Ethical aspects: The thesis protocol was approved by IRB committee, Faculty of Medicine, Zagazig University. Subjects and Methods: This study was carried on 40 adult patients with IPF. Patients were divided into:  Group (A): 20 patients accepted to be treated with pirfenidone.  Group (B): also 20 patients they have been treated with conventional treatment for IPF Results: Mean change in distance walked during 6MWD test was 0.05 ± 56.24 for group (A) and −12.65 ± 67.86 for group (B). Mean decline in predicted FVC was −0.89 %± 14.55 for group (A) and −5.48% ± 10.89 for group(B) there were progression of the disease according to HRCT in 60% in group(A) and 70% in group(B), , while only 10% in group (A) showed regression of the disease with statistical significant differences between two groups. Conclusion: Pirfenidone didn't improve health related quality of life in IPF patients, Pirfenidone did not affect oxygenation in treatment group after 3 months therapy , Pirfenidone has good impact on radiological presentation of IPF patients. Pirfenidone not only slow loss in FVC but also improved it in one third of treated patients, GIT upset is the main adverse effects of pirfendone which are mild.