TY - JOUR
T1 - A combined therapeutic approach in progressive idiopathic pulmonary fibrosis - Pirfenidone as bridge therapy for Ex vivo lung transplantation
T2 - A case report
AU - Paone, G.
AU - Sebastiani, A.
AU - Ialleni, E.
AU - Diso, D.
AU - Rose, D.
AU - Quagliarini, F.
AU - Ialongo, P.
AU - Peruzzi, M.
AU - Venuta, F.
AU - Frati, G.
PY - 2015/4/1
Y1 - 2015/4/1
N2 - 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.
AB - 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.
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U2 - 10.1016/j.transproceed.2015.02.006
DO - 10.1016/j.transproceed.2015.02.006
M3 - Article
C2 - 25891747
AN - SCOPUS:84928334935
VL - 47
SP - 855
EP - 857
JO - Transplantation Proceedings
JF - Transplantation Proceedings
SN - 0041-1345
IS - 3
ER -