Paone G, Sebastiani A, Ialleni E, Diso D, Rose D, Quagliarini F, Ialongo P, Peruzzi M, Venuta F, Frati G
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.
Department of Respiratory Diseases, S. Camillo-Forlanini Hospital, Rome, Italy.
Transplant Proc. 2015 Apr;47(3):855-7. 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.
特发性肺纤维化（IPF）是一种病因不明的进行性纤维化间质性肺炎，与普通型间质性肺炎（UIP）的组织病理学模式相关，并导致呼吸功能逐渐下降。本文描述了一例62岁的戒烟者因IPF转诊至我院的病例。经过2年的随访，该患者的肺功能显著恶化，并被纳入肺移植项目。此后，开始使用吡非尼酮进行药物治疗，实现了呼吸功能的稳定。根据多伦多模式，该患者通过常温体外肺灌注（EVLP）方法接受了单肺移植。在20个月的评估中，患者的呼吸功能显著改善，生活质量也得到了大幅提高。我们认为，综合多学科方法应被视为治疗IPF患者的关键选择。
