%0 Journal Article %A Raja Dhar %A K.S. Satish %A Sujeet Rajan %A R. Vijai Kumar %A Zarir Udwadia %A Harjit Dumra %A Murali Mohan %A Rahul Namjoshi %A Namita Ghag %A Jaideep Gogtay %T Clinical profile and early follow-up of patients receiving pirfenidone in the PIONEER observational study %D 2014 %J European Respiratory Journal %P P3792 %V 44 %N Suppl 58 %X BACKGROUND: PIONEER is an observational, PractIce based, Open label, Non-comparative, multicEnter study to Evaluate the efficacy, toleRability and safety of pirfenidone in idiopathic pulmonary fibrosis (IPF) patients from India.OBJECTIVES: To understand the clinical profile of patients prescribed pirfenidone therapy.METHODS: In this interim analysis, clinical profile of 90 patients on pirfenidone treatment is reported.RESULTS: Mean age was 60.95±11.13 years (46 males). Mean dose and duration of treatment on pirfenidone was 1031.06±360.2 mg and 29.26±20.00 weeks. Clinical features at baseline were dry cough 70 (77.78%), dyspnea on exertion 65 (72.22%), desaturation on exercise 26 (28.89%), clubbing 12 (13.33%), crackles 13 (14.44%) and crepitations/rales in 39 (43.33%) patients. High resolution computed tomography (HRCT) data was available in 88 patients, 62(70.45%) patients showed usual interstitial pneumonia (UIP)-IPF pattern and the rest had an “off-label prescription” of pirfenidone for non UIP-IPF pattern. Mean± SD baseline values of forced vital capacity (FVC) (n=74), six minute walk test (6MWT) (n=36) and diffusion capacity DLCO (n=36) were 1.58± 0.663 L, 365.79±130.65 meters and 48.32±26.76 %. Mean change± SD in FVC (n=13 with FVC data for 48 weeks) was 0.09±0.27 L. Most common adverse events reported (5 each) were gastrointestinal, skin-related and respiratory.CONCLUSION: We present the baseline data for patients on pirfenidone in the PIONEER study. In a small subgroup of patients who had FVC data at 24 and 48 weeks, pirfenidone appears to stabilize lung function. Further results from the study are awaited. %U