Pirfenidone treatment in idiopathic pulmonary fibrosis: nationwide Danish results.
BACKGROUND
Pirfenidone was approved by the European Medicines Agency and introduced in most European countries in 2011 for treatment of idiopathic pulmonary fibrosis (IPF).


OBJECTIVE
To describe the national Danish experiences of pirfenidone treatment for IPF during 30 months with respect to target population, safety, adherence to the treatment and effect analysis in a well-characterised IPF population in a real-life setting.


METHODS
Retrospective data collection from medical records of all patients in Denmark with IPF from 2011 to 2014. Data included baseline demographics, high-resolution computed tomography (HRCT), histopathology, forced vital capacity (FVC) and 6-min walk test (6MWT). Longitudinal data on FVC, walk test, adherence to the treatment and vital status were also collected.


RESULTS
Pirfenidone treatment was initiated in 113 patients. Mean age was 69.6±8.1 years (±SD), and 71% were male. Definite IPF diagnosis required thoracoscopic lung biopsy in 45 patients (39.8%). The remaining 68 cases had a definite (64 patients) or possible usual interstitial pneumonia (four patients) pattern on HRCT. Patients were followed for 0.1-33.8 months (median 9.4 months). Fifty-one patients (45.2%) needed dose adjustment, 18 (16%) patients discontinued therapy and 13 patients (11.5%) died. The annual mean decline in FVC was 164 ml (SE 33.2). The decline in 6MWT was 18.2 m (SE 11.2). Nausea (44.2%), fatigue (38.9%) and skin reactions (32.7%) were frequent adverse events.


CONCLUSIONS
Patients with IPF treated with pirfenidone experienced tolerable adverse events. Patients were maintained on treatment due to a careful follow-up and dose adjustment programme. The annual decline in physiological parameters and mortality rate was comparable to previous randomised controlled trials.
Department of Respiratory Medicine, Gentofte University Hospital, Hellerup, Denmark; goran@dadlnet.dk.
Goran Nadir
GN
Salih
Department of Respiratory Medicine, Gentofte University Hospital, Hellerup, Denmark.
Saher Burhan
SB
Shaker
Department of Respiratory Medicine, Odense University Hospital, Odense, Denmark.
Helle Dall
HD
Madsen
Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark.
Elisabeth
E
Bendstrup
efficacy; idiopathic pulmonary fibrosis; pirfenidone; safety; therapy