Abstract
Introduction
Idiopathic pulmonary fibrosis (IPF) is the most devastating form of idiopathic interstitial pneumonia. Encouraging results have led to licensing of the first IPF-specific drug, pirfenidone. The present study is an attempt to analyse the response of IPF patients to this treatment.
Aims and objectives
To know the proportion of IPF among interstitial lung diseases(ILD) in a tertiary care center in South India and their response to treatment.
Methods
Patients attending Institute of Chest Disease during the study period from January to June 2012 with a proven diagnosis of ILD were considered for a longitudinal single group cohort study. After detailed evaluation, 22 patients with IPF in functional class II & III were managed with Pirfenidone, N acetyl cystine (NAC), Proton pump inhibitors (PPI) and long term oxygen therapy (LTOT),if indicated. These patients were followed up with spirometry and 6 Minutes walk test once in 3 months and 6 monthly Diffusion capacity and high resolution computed tomography was repeated in selected cases.
Results
A total of 69 ILD patients attended our outpatient department during the study period which included 24 IPF patients. During the 12months follow up period 5 patients expired, among which 1 was due to acute coronary event and the remaining 4 due to respiratory failure. 4 patients in pirfenidone group remained relatively stable during the study period and all others deteriorated.
Conclusions
The prevalence of IPF among ILD in our hospital is 35% with female predominance. The present study does not show significant improvement in IPF patients on Pirfenidone, NAC, PPI combination with or without LTOT.
- © 2014 ERS