A Combined Therapeutic Approach in Progressive Idiopathic Pulmonary Fibrosis-Pirfenidone as Bridge Therapy for Ex Vivo Lung Transplantation: A Case Report

作者：Paone G^*; Sebastiani A; Ialleni E; Diso D; Rose D; Quagliarini F; Ialongo P; Peruzzi M; Venuta F; Frati G

来源：Transplantation Proceedings, 2015, 47(3): 855-857.

DOI：10.1016/j.transproceed.2015.02.006

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial pneumonia of unknown etiology, which is associated with the histopathologic pattern of usual interstitial pneumonia (UIP) and leads to a progressive decrease of respiratory function. The present article describes a case of a 62-year-old ex-smoker referred to our hospital because of IPF. After 2 years of follow-up, the subject experienced a significant worsening of pulmonary function and was enrolled in a lung transplantation program. Afterward, a pharmacological treatment with pirfenidone was started, achieving a stabilization of respiratory function. The patient underwent a single lung transplantation by means of a normothermic ex vivo lung perfusion (EVLP) approach according to the Toronto model. At 20-month evaluation the subject's respiratory function was significantly improved, and quality of life was considerably ameliorated. We believe that an integrated multidisciplinary approach should be considered a key option for the treatment of individuals with IPF.

• 出版日期2015-4