• Transplant. Proc. · Apr 2015

    Case Reports

    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, and G Frati.
    • Department of Cardiovascular, Respiratory, Nephrologic, Anesthesiologic and Geriatric Sciences, Sapienza University of Rome, Rome, Italy; Department of Respiratory Diseases, S. Camillo-Forlanini Hospital, Rome, Italy. Electronic address: rpaone1023@yahoo.com.
    • Transplant. Proc. 2015 Apr 1; 47 (3): 855-7.

    AbstractIdiopathic 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.Copyright © 2015 Elsevier Inc. All rights reserved.

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