Crohn's disease: an immune deficiency state.
Crohn's disease: an immune deficiency state.
复制标题
DOI：
10.1007/s12016-009-8133-2
复制
发表时间：
2010-02
影响因子：
9.1
通讯作者：
Segal AW
中科院分区：
文献类型：
--
作者：
Marks DJ;Rahman FZ;Sewell GW;Segal AW
克罗恩病是一种慢性炎症性疾病，主要影响胃肠道。其临床表现源于激活的白细胞对肠道粘膜的大量渗透和慢性炎症的下游后果。推动这种免疫反应的根本原因仍然知之甚少。已经提出了许多假说，其中大多数假说是基于活动性克罗恩病变的病理表现而提出的，其中大多数假说是免疫反应的初级过度激活。有趣的是，这些理论都没有得到机械证明。引发疾病的免疫学事件可能与导致疾病持续和传播的免疫事件有很大不同。近年来出现的大量数据表明，克罗恩病的主要缺陷实际上是相对免疫缺陷之一。这篇综述考虑了这种现象的证据，与其他流行的假说形成对比，并试图解决它所产生的一些潜在的悖论。
Crohn’s disease is a chronic inflammatory disorder primarily affecting the gastrointestinal tract. Its clinical manifestations arise from a substantial infiltration of the intestinal mucosa by activated leukocytes and the downstream consequences of chronic inflammation. The underlying cause driving this immunological reaction remains poorly understood. A number of hypotheses have been proposed, most of which postulate a primary over-activation of the immune response, based on the pathological appearances of active Crohn’s lesions. Interestingly, none of these theories have been mechanistically proven. It is possible that the immunological events responsible for disease initiation are quite different from those contributing to its persistence and propagation. A substantial body of data has emerged in recent years to suggest that the primary defect in Crohn’s disease is actually one of relative immunodeficiency. This review considers the evidence for such a phenomenon in contrast to alternative prevailing hypotheses and attempts to address some of the potential paradoxes that it generates.