Background Idiopathic pulmonary fibrosis (IPF) is a progressive idiopathic interstitial lung disease with a poor prognosis.
Patients with IPF have a poor quality of life and a median survival of about 3 years.
In the past years there was a breakthrough in the treatment of IPF. Pirfenidone and nintedanib are now approved for the treatment of IPF. Although nintedanib is not yet marketed in the European Union, the manufacturing laboratory has an extended programme that allows its use.
Pirfenidone and nintedanib are indicated for mild to moderate IPF.
Purpose To evaluate the effectiveness and safety of pirfenidone and nintedanib in patients with IPF.
Material and methods A retrospective observational analysis of the use of pirfenidone and nintedanib in our hospital from 2014 to October 2015 was conducted.
Variables included demographic (age, sex) and clinical data (previous treatment, side effects and clinical outcome). Adverse drug reaction (ADRs) were compiled in relation to safety.
Results 8 patients were included in the study (6 men and 2 women) with a mean age of 69 years.
5 patients were treated with pirfenidone; 2 of them stopped and continued a secondline treatment with nintedanib, 1 because of phototoxicity after 8 months of taking pirfenidone and the other because of significant deterioration in forced vital capacity (FVC).
These 5 patients did not present with digestive disturbances or an increase in transaminases.
5 patients received nintedanib, two of them as a secondline and 3 as a firstline treatement; 1 could not receive pirfenidone due to a glomerular filtration rate <30 mL/min.
2 patients had to reduce the dosage to 100 mg twice daily due to digestive disturbances (nausea and diarrhoea) and 1 had to discontinue treatment.
Only 2 patients did not present with any digestive disturbances or increase in transaminases.
Only 2 patients have been receiving treatment long enough to have follow-up data, 1 for pirfenidone and 1 for nintedanib. After 6 months of treatment, FVC had a less than 10% decrease (4% and 5%, respectively) and diffusing capacity or transfer factor of the lung for carbon monoxide (DLCO) increased by 1% each.
Conclusion Due to the short follow-up period, we cannot yet establish effectiveness.
ADRs caused discontinuation of treatment in two patients so close monitoring is required.
No conflict of interest.
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