Laporta Hernandez Rosalía, Aguilar Perez Myriam, Lázaro Carrasco María Teresa, Ussetti Gil Piedad
Hospital Universitario Puerta de Hierro de Majadahonda Madrid, 28222 Madrid, Spain.
Med Sci (Basel). 2018 Aug 23;6(3):68. doi: 10.3390/medsci6030068.
Despite the advances in recent years in the treatment of idiopathic pulmonary fibrosis (IPF), it continues to be a progressive disease with poor prognosis. In selected patients, lung transplantation may be a treatment option, with optimal results in survival and quality of life. Currently, pulmonary fibrosis is the main cause of lung transplantation. However, mortality on the waiting list of these patients is high, since many patients are referred to the transplant units with advanced disease. There is not a parameter that can predict the survival of a specific patient. Different variables are to be considered in order to decide the right time to send them to a transplant unit. It is also very difficult to decide when to include these patients on the waiting list. Every patient diagnosed with IPF, without contraindications for surgery, should be referred early to a transplant unit for assessment. A uni or bilateral transplantation will be decided based on the characteristics of the patient and the experience of each center. The post-transplant survival of recipients with IPF is lower than that observed in other diseases, such as cystic fibrosis or chronic obstructive pulmonary disease as a consequence of their older age and the frequent presence of associated comorbidity. Post-transplant follow-up must be tight in order to assure optimal level of immunosuppressive treatment, detect complications associated with it, and avoid graft rejection. The main cause of long-term mortality is late graft dysfunction as a consequence of chronic rejection. Other complications, such as infections and tumors, must be considered.
尽管近年来特发性肺纤维化(IPF)的治疗取得了进展,但它仍然是一种预后不良的进行性疾病。对于部分患者,肺移植可能是一种治疗选择,在生存和生活质量方面可取得最佳效果。目前,肺纤维化是肺移植的主要原因。然而,这些患者在等待名单上的死亡率很高,因为许多患者在疾病晚期才被转诊至移植单位。没有一个参数能够预测特定患者的生存情况。为了确定将患者送往移植单位的合适时机,需要考虑不同的变量。决定何时将这些患者列入等待名单也非常困难。每个诊断为IPF且无手术禁忌症的患者都应尽早转诊至移植单位进行评估。将根据患者的特征和各中心的经验决定进行单肺或双肺移植。由于年龄较大且常伴有合并症,IPF受者移植后的生存率低于囊性纤维化或慢性阻塞性肺疾病等其他疾病。移植后必须进行密切随访,以确保免疫抑制治疗达到最佳水平,检测与之相关的并发症,并避免移植物排斥反应。长期死亡的主要原因是慢性排斥导致的晚期移植物功能障碍。还必须考虑其他并发症,如感染和肿瘤。