Background Idiopathic pulmonary fibrosis (IPF) is a chronic, limited lung fibrosing interstitial pneumonia of unknown cause with poor prognosis and few therapeutic options, with a median survival of 2–5 years from diagnosis. It is characterised by fibroblast proliferation and abnormal accumulation of extracellular matrix molecules, particularly collagen fibres. Pirfenidone is the first IPF-specific antifibrotic, with anti-inflammatory and antifibrotic properties whose mechanism of action has not been fully established. To date, pirfenidone is the only drug with proven efficacy in the treatment of IPF. It is considered an orphan drug, and is not yet marketed in all European countries, so that additional monitoring is required.
Purpose To describe the progress of a patient treated with pirfenidone as well as the safety of this new treatment.
Material and methods A prospective observational study was conducted. The patient, a 65-year-old male, stopped smoking 2 years ago (with a cumulative tobacco consumption index of 57 packs/year). He was diagnosed with IPF in 2012 by clinical and radiological criteria. Pirfenidone was approved as a foreign drug for a period of three months by the Ministry of Health.
Results After titration, the usual pirfenidone dose was administered (2403 mg daily). After 3 months of treatment, forced vital capacity (FVC) experienced less than 10% decrease (only 1.2%) and diffusing capacity or transfer factor of the lung for carbon monoxide (DLCO) decreased by 6.1% showing no radiological progression. There were no increase in transaminases, no digestive disturbances or weight loss.
Conclusion Pirfenidone has been used successfully to date in our case, so it is possible to continue the treatment until a new patient evaluation at 6 months in order to prevent a lung transplant. Although more and longer treatment periods are needed, pirfenidone seems to be a well-tolerated treatment option for IPF.
References and/or Acknowledgements
References and/or AcknowledgementsNo conflict of interest.
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