Praprotnik Sonja, Ferluga Dusan, Vizjak Alenka, Hvala Anastazija, Avcin Tadej, Rozman Blaz
Clinical Department of Rheumatology, University Medical Center Ljubljana, Ljubljana, Slovenia.
Clin Rev Allergy Immunol. 2009 Jun;36(2-3):109-25. doi: 10.1007/s12016-008-8104-z.
The paper presents an overview of clinical manifestations and histopathologic findings in different organs in microvascular thrombotic and microangiopathic antiphospholipid syndrome (MAPS). Subsets of antiphospholipid syndrome (APS) are presented and defined. Clinico-pathologic correlations seem insufficient so far, because of a lack of detailed systematic studies of the histopathology in different organs. Based on their own autopsy and biopsy studies, the authors propose a novel categorization of histopathologic lesions that occur in patients with classic and catastrophic APS. In addition to the already accepted category of a microvascular thrombotic type of lesions, microangiopathic lesions consistent with thrombotic microangiopathy are proposed to be included in new revised classification criteria for definite APS. Microvascular thrombotic and so far underestimated microangiopathic histopathologic lesions have been shown to appear in various combinations and of different ages in patients with both classic and catastrophic APS, which fits into the concept of MAPS. These preliminary findings of our studies are also in line with the most recent hypothesis of two main mechanisms in the pathogenesis of APS, emphasizing a key role of endothelial cell affection induced by aPL on the one hand and interference with coagulation cascade on the other side.
本文概述了微血管血栓形成性和微血管病性抗磷脂综合征（MAPS）不同器官的临床表现和组织病理学发现。介绍并定义了抗磷脂综合征（APS）的亚型。由于缺乏对不同器官组织病理学的详细系统研究，目前临床病理相关性似乎不足。基于他们自己的尸检和活检研究，作者提出了一种对经典型和灾难性APS患者中出现的组织病理学病变的新分类。除了已被认可的微血管血栓形成性病变类别外，与血栓性微血管病一致的微血管病性病变被提议纳入确定性APS的新修订分类标准。微血管血栓形成性和迄今被低估的微血管病性组织病理学病变已被证明在经典型和灾难性APS患者中以各种组合和不同年龄出现，这符合MAPS的概念。我们研究的这些初步发现也与APS发病机制中两个主要机制的最新假说一致，一方面强调抗磷脂抗体（aPL）诱导的内皮细胞损伤的关键作用，另一方面强调对凝血级联反应的干扰。
