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Study of overall survival associated with nintedanib and pirfenidone in patients with idiopathic pulmonary fibrosis: a real-life comparison

Idiopathic pulmonary fibrosis (IPF) is a rare disease with a very debilitating course for which only limited therapeutic options are available. The incidence of IPF is increasing worldwide and in Italy ranges between 7.5 and 9.3 new cases per 100 000 per year. It is characterized by progressive and irreversible scarring of lung tissue resulting in a decline in respiratory function and usually a fatal outcome; survival of patients with IPF is usually less than 5 years from diagnosis. Currently, pharmacological treatments include only two active ingredients to delay disease progression: nintedanib, a tyrosine kinase inhibitor, and pirfenidone, a molecule with antiinflammatory and antifibrotic activities. A retrospective analysis was conducted by hospital pharmacy staff at a Lombard Rare Disease Network (LRDN) Centre to assess overall survival (OS) associated with nintedanib and pirfenidone in real life, with the aim of comparing the effectiveness of the two medicines in real practice and collecting data to support physicians’ therapeutic choices. The review involved all patients treated with nintedanib and pirfenidone from 1 January 2015 to 30 June 2021. Data were collected using the hospital pharmacy management software (Oracle) and through informations provided by physicians. No formal ethics committee approval was necessary because pulmonologists provided strictly anonymous patient data. The analysis did not come under the Human Research Act. Ninetytwo patients were treated during the period analyzed. Twelve were excluded from the study, six because they had moved to other centres and six whose treatment was interrupted because of severe gastrointestinal side effects. The 80 patients assessed (17 women and 63 men, mean age 75 years) were divided into two groups of 40, treated with nintedanib and pirfenidone respectively. Overall, 18 patients (16 men and 2 women, mean age 76 years) died due to the progression of the disease: 5 of these were intaking nintedanib and 13 received pirfenidone. On average, reallife data show an OS of 35 months, with a median value of 33 months. The results of the analysis do not highlight significant differences between the two groups; indeed, patients treated with nintedanib had a mean OS of 31 months and a median value of 25 months, while patients treated with pirfenidone had a mean OS of 36 months and a median of 38 months (figure 1). These results are slightly lower than data extracted from the literature, which currently show an average OS of 44 months, probably due to the smaller sample size of our analysis. 5 According to the medical team, an increase in the incidence of IPF in older patients is expected in the coming years, therefore this study could be conducted on a larger sample involving all LRDN centres to support the preliminary data collected with major evidence. Thus, it would be desirable that hospital pharmacists continue to support clinical assessments in setting up pharmacological treatments in patients with IPF by extending the research to other LRDN centres and collaborating in multidisciplinary teams to increase the depth of knowledge about this rare disease.