A combined therapeutic approach in progressive idiopathic pulmonary fibrosis-pirfenidone as bridge therapy for ex vivo lung transplantation: a case report.

Abstract

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.

• Authors: G Paone, A Sebastiani, E Ialleni, D Diso, D Rose, F Quagliarini, P Ialongo, M Peruzzi, F Venuta, G Frati
• Journal: Transplantation proceedings (Transplant Proc) Vol. 47 Issue 3 Pg. 855-7 (Apr 2015) ISSN: 1873-2623 [Electronic] United States
• PMID: 25891747 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2015 Elsevier Inc. All rights reserved.

Chemical References

• Anti-Inflammatory Agents, Non-Steroidal
• Pyridones
• pirfenidone

Topics

• Anti-Inflammatory Agents, Non-Steroidal (therapeutic use)
• Combined Modality Therapy
• Disease Progression
• Humans
• Idiopathic Pulmonary Fibrosis (drug therapy, surgery)
• Lung Transplantation
• Male
• Middle Aged
• Pulmonary Fibrosis (therapy)
• Pyridones (therapeutic use)
• Quality of Life