Division of Pulmonary and Critical Care, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Curr Opin Rheumatol. 2018 Nov;30(6):562-569. doi: 10.1097/BOR.0000000000000546.
The purpose of this review is to highlight recent data regarding feasibility and outcomes following lung transplantation for patients with systemic sclerosis related pulmonary disease as well as to emphasize areas of uncertainly in need of further study. We include a description of our centre's approach to lung transplant evaluation and posttransplant management in this complex patient population.
Historical data have demonstrated that patients with scleroderma have an increased risk of complications following lung transplantation owing to the multisystem nature of disease, particularly concurrent gastrointestinal, cardiac and renal involvement. Emerging data support the safety of lung transplant in appropriately selected patients with scleroderma-related interstitial lung disease and pulmonary arterial hypertension.
Accumulating evidence validates that a diagnosis of scleroderma is not a priori a contraindication to lung transplant. In the carefully selected patient, both short-term and long-term outcomes following lung transplantation are comparable to counterparts with fibrotic lung disease or pulmonary arterial hypertension. However, further prospective study to detail how these patients should be evaluated and managed posttransplant is definitely needed. Cardiac disease is an emerging cause of morbidity and mortality in the scleroderma population and deserves particular attention during the pre and posttransplant period.
本文旨在强调有关系统性硬化症相关肺部疾病患者肺移植的可行性和结果的最新数据，并强调需要进一步研究的不确定领域。我们在本文中描述了我们中心在这一复杂患者群体中进行肺移植评估和移植后管理的方法。
历史数据表明，由于疾病的多系统性质，特别是同时存在胃肠道、心脏和肾脏受累，系统性硬化症患者在肺移植后发生并发症的风险增加。新出现的数据支持在适当选择的系统性硬化症相关间质性肺疾病和肺动脉高压患者中进行肺移植的安全性。
越来越多的证据证实，系统性硬化症的诊断并非肺移植的绝对禁忌证。在经过精心选择的患者中，肺移植后的短期和长期结果与纤维化肺部疾病或肺动脉高压患者相当。然而，确实需要进一步的前瞻性研究来详细说明如何对这些患者进行评估和移植后管理。心脏疾病是系统性硬化症患者发病率和死亡率的一个新出现原因，在移植前和移植期间都需要特别注意。
