Le Pavec Jérôme, Dauriat Gaëlle, Gazengel Pierre, Dolidon Samuel, Hanna Amir, Feuillet Séverine, Pradere Pauline, Crutu Adrian, Florea Valentina, Boulate David, Mitilian Delphine, Fabre Dominique, Mussot Sacha, Mercier Olaf, Fadel Elie
Service de chirurgie thoracique, vasculaire et transplantation cardio-pulmonaire, hôpital Marie-Lannelongue, Le Plessis-Robinson, France; Faculté de médecine, université Paris-Sud, université Paris-Saclay, Le Kremlin Bicêtre, France; UMR_S 999, Inserm, université Paris-Sud, hôpital Marie-Lannelongue, Le Plessis-Robinson, France.
Service de chirurgie thoracique, vasculaire et transplantation cardio-pulmonaire, hôpital Marie-Lannelongue, Le Plessis-Robinson, France; Faculté de médecine, université Paris-Sud, université Paris-Saclay, Le Kremlin Bicêtre, France; UMR_S 999, Inserm, université Paris-Sud, hôpital Marie-Lannelongue, Le Plessis-Robinson, France.
Presse Med. 2020 Jun;49(2):104026. doi: 10.1016/j.lpm.2020.104026. Epub 2020 May 11.
Idiopathic pulmonary fibrosis (IPF) is characterized by relentlessly progressive lung function impairment that is consistently fatal in the absence of lung transplantation, as no curative pharmacological treatment exists. The pace of progression varies across patients, and acute life-threatening exacerbations occur unpredictably, causing further sharp drops in lung function. Recently introduced antifibrotic agents slow the pace of disease progression and may improve survival but fail to stop the fibrotic process. Moreover, the magnitude and kinetics of the response to these drugs cannot be predicted in the individual patient. These characteristics require that lung transplantation be considered early in the course of the disease. However, given the shortage of donor lungs, lung transplantation must be carefully targeted to those patients most likely to benefit. Current guidelines for lung transplantation listing may need reappraisal in the light of recent treatment advances. Patients with IPF often have multiple comorbidities such as coronary heart disease, frailty, and gastro-oesophageal reflux disease (GERD). Consequently, extensive screening for and effective treatment of concomitant conditions is crucial to appropriate candidate selection and outcome optimisation. A multidisciplinary approach is mandatory. Pulmonologists with expertise in IPF must work closely with lung transplant teams. Careful consideration must be given to preoperative optimisation, surgical technique, and pulmonary rehabilitation to produce the best post-transplantation outcomes.
特发性肺纤维化（IPF）的特点是肺功能持续进行性损害，在没有肺移植的情况下通常会导致死亡，因为目前尚无治愈性的药物治疗方法。疾病进展速度因患者而异，并且会不可预测地发生危及生命的急性加重，导致肺功能进一步急剧下降。最近引入的抗纤维化药物可减缓疾病进展速度，并可能改善生存率，但无法阻止纤维化进程。此外，无法在个体患者中预测对这些药物的反应程度和动力学。这些特点要求在疾病进程早期就考虑肺移植。然而，鉴于供体肺短缺，肺移植必须谨慎地针对最有可能受益的患者。鉴于最近的治疗进展，目前的肺移植列入标准指南可能需要重新评估。IPF患者常伴有多种合并症，如冠心病、身体虚弱和胃食管反流病（GERD）。因此，对伴随疾病进行广泛筛查和有效治疗对于合适的候选者选择和优化治疗结果至关重要。必须采取多学科方法。擅长IPF的肺科医生必须与肺移植团队密切合作。必须仔细考虑术前优化、手术技术和肺康复，以获得最佳的移植后效果。
