Bronchiolitis obliterans syndrome: the Achilles' heel of lung transplantation.
Bronchiolitis obliterans syndrome: the Achilles' heel of lung transplantation.
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DOI：
10.1055/s-0033-1348467
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发表时间：
2013-06
影响因子：
3.2
通讯作者：
Belperio JA
中科院分区：
文献类型：
--
作者：
Weigt SS;DerHovanessian A;Wallace WD;Lynch JP 3rd;Belperio JA
肺移植是终末期肺部疾病患者的治疗选择。不幸的是，慢性肺移植物功能障碍（CLAD），最常见的表现为闭塞性细支气管炎综合征（BOS），仍然是非常普遍的，是长期生存的主要限制。BOS的发病机制复杂，涉及同种免疫和非同种免疫途径。在临床上，BOS表现为气道阻塞和呼吸困难，典型的进展性和最终致命的;然而，过程是高度可变的，可区分的表型可能存在。有几个对照研究评估治疗效果，但只有少数患者响应当前的治疗方式。最终，预防性策略可能被证明在延长肺移植后的生存期方面更有效，但关于预防BOS的最佳策略仍存在相当大的争议和很少的数据。更好地了解慢性肺移植排斥反应的危险因素及其与病理机制的关系，将导致更好的药理学靶点，以预防或治疗这种综合征。
Lung transplantation is a therapeutic option for patients with end-stage pulmonary disorders. Unfortunately, chronic lung allograft dysfunction (CLAD), most commonly manifest as bronchiolitis obliterans syndrome (BOS), continues to be highly prevalent and is the major limitation to long-term survival. The pathogenesis of BOS is complex and involves alloimmune and nonalloimmune pathways. Clinically, BOS manifests as airway obstruction and dyspnea that are classically progressive and ultimately fatal; however, the course is highly variable, and distinguishable phenotypes may exist. There are few controlled studies assessing treatment efficacy, but only a minority of patients respond to current treatment modalities. Ultimately, preventive strategies may prove more effective at prolonging survival after lung transplantation, but their remains considerable debate and little data regarding the best strategies to prevent BOS. A better understanding of the risk factors and their relationship to the pathological mechanisms of chronic lung allograft rejection should lead to better pharmacological targets to prevent or treat this syndrome.