狼疮性肾炎合并灾难性抗磷脂抗体综合征的临床研究综述及病例报告

M. Stoian, Gabriel Scarlat, S. Seitan, Bianca Procopiescu
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摘要

摘要肾小球肾炎是系统性红斑狼疮(SLE)发病的主要原因。事实上,免疫复合物在肾脏中的形成和沉积导致肾小球内炎症,白细胞募集,固有肾细胞活化和增殖。严重损伤可通过坏死或细胞凋亡破坏常驻肾细胞,导致纤维蛋白样坏死。当损伤程度较低时,毛细血管内细胞通过增殖和产生细胞外基质(增殖性病变)作出反应。肾活检、尿沉渣检查和C3水平测量(以及降低抗DNA滴度)对于狼疮性肾炎的治疗至关重要。抗磷脂综合征(APS)是一种全身性自身免疫性疾病,其特征是在存在抗磷脂抗体(aPL)(即狼疮抗凝血剂(LA)、抗心磷脂抗体和抗β2糖蛋白-I抗体(抗β2GPI))的情况下,动脉或静脉循环中出现血栓。灾难性APS(CAPS)是一种非常罕见(---lt---1%)且极其严重的APS变体。其特征是在很短的时间内(几天到几周)发生多个系统和血栓性器官受累。肾脏受累是CAPS的常见特征,最常见的发现是血栓性微血管病(TMA),但也可以发现APSN的其他慢性病变。
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A Clinical Approach of Lupus Nephritis Associated with Catastrophic Antiphospholipid - Antibody Syndrome - Review and Case Report
Abstract Glomerulonephritis is a major cause of morbidity in systemic lupus erythematosus (SLE). In fact, immune complex formation and deposition in the kidney results in intraglomerular inflammation with recruitment of leukocytes and activation and proliferation of resident renal cells. Intense injury may destroy resident renal cells by necrosis or apoptosis resulting in fibrinoid necrosis. When injury is less intense, endocapillary cells respond by proliferating and production of extracellular matrix (proliferative lesions). Renal biopsy, examination of urine sediment and measurement of C3 levels (and to less anti-DNA titers) are essential for the management of lupus nephritis. Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by thrombotic episodes in the arterial or venous circulation, in the presence of antiphospholipid antibodies (aPL), namely lupus anticoagulant (LA), anticardiolipin antibodies, and anti-β2glycoprotein-I antibodies (anti-β2GPI). Catastrophic APS (CAPS) is a very rare (---lt---1%) and extremely severe variant of APS. It is characterized by multiple systems and thrombotic organ involvement that occurs in a very short period (days to weeks). Renal involvement is a common feature in CAPS, the most frequent finding is thrombotic microangiopathy (TMA), but other chronic lesions of APSN can also be found.
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