A combined therapeutic approach in progressive idiopathic pulmonary fibrosis - Pirfenidone as bridge therapy for Ex vivo lung transplantation: A case report

G. Paone, A. Sebastiani, E. Ialleni, D. Diso, D. Rose, F. Quagliarini, P. Ialongo, M. Peruzzi, F. Venuta, G. Frati

Research output: Contribution to journalArticle


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.

Original languageEnglish
Pages (from-to)855-857
Number of pages3
JournalTransplantation Proceedings
Issue number3
Publication statusPublished - Apr 1 2015


ASJC Scopus subject areas

  • Surgery
  • Transplantation
  • Medicine(all)

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