Background Idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial pneumonia of unknown origin with a poor prognosis. Pirfenidone has shown inhibitory activity of fibroblast proliferation and collagen synthesis in vitro. In some clinical assays the drug has been shown to slow the progression of disease. Although it has not demonstrated improvement in overall survival, pirfenidona is the first specific drug therapy for IPF.
Purpose To evaluate the effectiveness and safety of treatment with pirfenidone in patients with mild to moderate IPF.
Material and methods A retrospective observational study from October 2014 to October 2015. Clinical data were obtained by medical record review. The main clinical variable studied was the variation in forced vital capacity (FVC). In fact, this variable was the main parameter of the CAPACITY study, which allowed authorisation of the drug. Data were available for respiratory function at the beginning and after pirfenidone treatment. Other variables such as forced expiratory volume (FEV1), diffusion capacity of the lung for carbon monoxide (DLCO) and desaturation at the end of the 6 min walking test (6MW) were collected. Treatment failure was considered a decrease of >10% in FVC. Safety was assessed by collecting all adverse events (AE) that occurred during treatment.
Results 8 patients, mean age 72 (55–83) years, 75% male, were included in our study during the past year. 5 patients showed increased FVC (+7% (1–11%)) and 3 showed decreased FVC (-6% (-1–15%)). Other variables studied (FEV1, 6MWT and DLCO) were not recorded for all patients. However, 2 patients with available data showed improvement in 6MWT and a decrease in DLCO. AE detected were: increased transaminase levels (1 patient), diarrhoea and dyspnea (1), anorexia (1) and photosensitivity (1). Only patient who suffered photosensitivity suspended treatment temporarily.
Conclusion Most patients showed a slowdown in the loss of FVC and improvement at the end of the 6MWT desaturation; only one patient had treatment failure.
AE were mild and similar to those described in the literature.
More studies are required to evaluate the benefit and to assess whether this slight improvement in FVC is related to improvement in the quality of life.
No conflict of interest.
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