Hoxha Ariela, Del Prete Dorella, Condonato Irene, Martino Francesca K, Lovisotto Marco, Nalesso Federico, Simioni Paolo
Internal Medicine Unit, Thrombotic and Hemorrhagic Center, Department of Medicine-DIMED, University of Padua, Via Giustiniani 2, 35128 Padova, Italy.
Nephrology Unit, Department of Medicine, University of Padua, 35128 Padua, Italy.
J Clin Med. 2025 May 10;14(10):3326. doi: 10.3390/jcm14103326.
Antiphospholipid syndrome (APS) can affect the kidneys, leading to renal artery and vein thrombosis, allograft loss following transplantation, and microvascular damage referred to as aPL-nephropathy (aPL-N). APL-N is a complex and frequently underdiagnosed condition characterized by an incomplete understanding of its etiopathogenesis and associated with unfavorable renal outcomes. The 2023 ACR/EULAR classification criteria for APS included aPL-N within the microvascular domain. The gold standard for aPL-N is the biopsy, revealing lesions associated with acute thrombotic microangiopathy and chronic vascular changes. Nevertheless, reluctance for biopsies due to anticoagulation and thrombocytopenia underscores the need for noninvasive diagnostics. Common clinical features include hypertension, microscopic hematuria, proteinuria, and renal insufficiency. Antiphospholipid antibodies seem crucial to kidney damage through thrombotic and inflammatory processes. Studies and experimental models of thrombotic microangiopathy lesions suggest the involvement of the complement cascade, tissue factor, and mammalian target of the rapamycin complex activation pathway. Currently, the management of aPL-N is based mainly on expert opinion, with limited evidence supporting the use of anticoagulants, leading to controversy in their application. Treatment may include heparin, intravenous immunoglobulin, plasma exchange, and targeted therapies tailored to aPL-N mechanisms. Future multicenter studies are essential to clarify their roles. The goal of this review is to inform clinicians and create a research agenda to address the unmet needs in diagnosing and managing APL-N.
抗磷脂综合征（APS）可累及肾脏，导致肾动静脉血栓形成、移植后同种异体肾丢失以及被称为抗磷脂抗体相关性肾病（aPL-N）的微血管损伤。aPL-N是一种复杂且常被漏诊的疾病，其病因发病机制尚未完全明了，且与不良肾脏预后相关。2023年美国风湿病学会（ACR）/欧洲抗风湿病联盟（EULAR）的APS分类标准将aPL-N纳入微血管病变范畴。aPL-N的金标准是活检，可发现与急性血栓性微血管病及慢性血管改变相关的病变。然而，由于抗凝和血小板减少而不愿进行活检，凸显了对无创诊断方法的需求。常见临床特征包括高血压、镜下血尿、蛋白尿和肾功能不全。抗磷脂抗体似乎通过血栓形成和炎症过程对肾脏损伤起关键作用。血栓性微血管病病变的研究及实验模型提示补体级联反应、组织因子和雷帕霉素复合物哺乳动物靶点激活途径参与其中。目前，aPL-N的治疗主要基于专家意见，支持使用抗凝剂的证据有限，这导致其应用存在争议。治疗可能包括肝素、静脉注射免疫球蛋白、血浆置换以及针对aPL-N机制的靶向治疗。未来的多中心研究对于阐明它们的作用至关重要。本综述的目的是为临床医生提供信息，并制定一个研究议程，以满足aPL-N诊断和管理方面未被满足的需求。
