Abstract
Background: Pirfenidone is the first therapy for idiopathic pulmonary fibrosis (IPF) that alters the natural progression of the disease. We report our experience thus far, on Pirfenidone use at a specialist interstitial lung disease clinic in Ireland.
Aims: We prospectively evaluated the tolerability and change in lung function before and after Pirfenidone therapy.
Methods: Consecutive patients on treatment with mild/moderate IPF (FVC≥50% predicted, DLCO≥35% predicted) were included. Patient demographics, pirfenidone dosing regimen, tolerability, side effect profile and pulmonary function tests were recorded from chart review.
Results: Study included 51 patients on Pirfenidone, of which 35 were males. The mean age was 72.5yrs (56-88yrs). The mean FVC% (L) and DLCO% (ml/min/mmHg) before and after treatment were 83.30(2.62) vs. 77.60(2.45) and 44.80(7.2) vs. 41.4(6.76).The mean duration of therapy was 10.6 months. The mean decline in FVC was 216.7ml compared to 235ml for placebo group in ASCEND study1 (p value 0.009). 49 patients are still alive and 33 (65%) tolerated maximal dose, 14 (27%) patients were on reduced dose due to side-effects and 2 (4%) patients discontinued therapy. Side effects were reported by 24 (47%) patients on pirfenidone treatment, of which, nausea 38%, diarrhoea 25%, rash 17%, dizziness 13%, myalgia 8% and 13% had abnormal liver profile.
Conclusions: The data suggest that pirfenidone therapy was well tolerated and effective in a real life setting and compares well to the ASCEND data.
Reference:
King TE Jr et al.ASCEND study group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2083-92.
- Copyright ©ERS 2015