Efficacy of pirfenidone in idiopathic pulmonary fibrosis: A single center experience
2016
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease with an estimated median survival of only 3 years after diagnosis. Recent trials involving pirfenidone and nintedanib show a beneficial effect in the treatment of mild-to-moderate idiopathic pulmonary fibrosis. Some patients however seem to improve after start of antifibrotics. OBJECTIVE: To assess lung functional evolution of patients, diagnosed with IPF, who started treatment with pirfenidone at a tertiary care hospital in Leuven, Belgium. METHODS: Pulmonary function measurements of 99 patients diagnosed with mild-to-moderate IPF who started on pirfenidone were retrospectively collected from start of treatment until 12 months of follow up at a 3-month interval. During the study period, we compared % predicted forced vital capacity (%FVC) and % predicted diffusing capacity of the lungs for carbon monoxide (%DLCO), RESULTS: An increase in %FVC of 5% was seen in 14 of the 99 patients (14.1%) and 16 patients (16.1%) at 6 and 12 months respectively. An increase with 10% in %FVC was seen 6 patients (6.1%) at 6 months and 10 patients (10.1%) at 12 months. Stable disease, defined as change in %FVC between 5 and -5%, was seen in 37 (37.1%) patients and 25 (25.1%) patients at 6 and 12 months respectively. A similar result was seen in % DLCO as 8 of the 99 patients (8.1%) showed an increased %DLCO at the 6-month interval of more than 15%. That number increased up to 10 patients (10.1%) after 12 months of treatment. CONCLUSION: These data suggest that there is a subgroup of patients who improve on pirfenidone treatment. However, further research is necessary to identify those responders.
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