Shah R, Brodsky S V, Hebert L, Rovin B H, Nadasdy T, Satoskar A A
1 Department of Internal Medicine, Division of Nephrology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
2 Department of Pathology, Division of Renal and Transplant Pathology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
Lupus. 2018 Dec;27(14):2236-2244. doi: 10.1177/0961203318809177. Epub 2018 Nov 7.
Antiphospholipid antibody syndrome (APS) is an acquired prothrombotic autoimmune disease caused by the presence of antibodies against anionic phospholipids or plasma proteins bound to phospholipids on cell membranes. It can be a primary disease or secondary to other autoimmune diseases, most commonly systemic lupus erythematosus (SLE). Laboratory testing for antiphospholipid antibodies (aPL) may be only transiently positive, so APS could be missed until a catastrophic thrombotic episode or pregnancy morbidity occurs. In the kidneys, this manifests as thrombotic microangiopathy (TMA), and patients present with hypertensive urgency and acute kidney injury. However, APS may not always have a catastrophic presentation but instead a more smoldering course. Kidney biopsy may not show obvious active TMA lesions but rather only chronic injury in the form of zonal cortical scarring and tubular thyroidization. Still, it may warrant anticoagulation therapy. So it is important to recognize this pattern of injury in the biopsy. Herein, we retrospectively study the correlation between presence of this histologic feature in kidney biopsies of SLE patients and positive aPL testing results (anticardiolipin antibodies and/or lupus anticoagulant). Kidney biopsies of SLE patients from 2004 to 2015 ( n = 186) were screened for presence or absence of zonal cortical scarring. Their electronic medical records were reviewed for aPL results. Our study showed low sensitivity (33%) but higher positive predictive value (62%), specificity (89%) and negative predictive value (71%). This histologic finding is therefore not a sensitive screening tool, but if present, greatly increases the likelihood of underlying aPL. We want to emphasize that recognition of this histologic feature in the biopsies of SLE patients is important so as not to miss the opportunity to treat with anticoagulation therapy and possibly slow down the chronic renal damage.
抗磷脂抗体综合征（APS）是一种获得性血栓形成性自身免疫性疾病，由针对阴离子磷脂或细胞膜上与磷脂结合的血浆蛋白的抗体引起。它可以是原发性疾病，也可以继发于其他自身免疫性疾病，最常见的是系统性红斑狼疮（SLE）。抗磷脂抗体（aPL）的实验室检测可能只是短暂阳性，因此在发生灾难性血栓事件或妊娠并发症之前，APS可能会被漏诊。在肾脏中，这表现为血栓性微血管病（TMA），患者表现为高血压急症和急性肾损伤。然而，APS并不总是表现为灾难性发作，而是可能有一个更为隐匿的病程。肾活检可能不会显示明显的活动性TMA病变，而仅表现为以带状皮质瘢痕和肾小管甲状腺样变为形式的慢性损伤。尽管如此，仍可能需要抗凝治疗。因此，在活检中识别这种损伤模式很重要。在此，我们回顾性研究SLE患者肾活检中这种组织学特征的存在与aPL检测阳性结果（抗心磷脂抗体和/或狼疮抗凝物）之间的相关性。对2004年至2015年SLE患者的肾活检（n = 186）进行筛选，以确定是否存在带状皮质瘢痕。查阅他们的电子病历以获取aPL结果。我们的研究显示敏感性较低（33%），但阳性预测值较高（62%）、特异性（89%）和阴性预测值（71%）。因此，这种组织学发现不是一种敏感的筛查工具，但如果存在，则大大增加了潜在aPL的可能性。我们想强调的是，在SLE患者的活检中识别这种组织学特征很重要，以免错过抗凝治疗的机会，并可能减缓慢性肾损伤。
