Internal Medicine Rheumatology Service, Centro Médico ABC, Mexico City, Mexico.
Nephrology Department, Centro Medico ABC, Mexico City, Mexico.
Front Immunol. 2018 May 17;9:1008. doi: 10.3389/fimmu.2018.01008. eCollection 2018.
Antiphospholipid syndrome is a complex autoimmune disease, characterized by the presence of vascular thrombosis, obstetric, hematologic, cutaneous, and cardiac manifestations. Renal disease in patients with antiphospholipid syndrome was not recognized in the first descriptions of the disease, but later on, the renal manifestations of the syndrome have been investigated widely. Renal manifestations of antiphospholipid syndrome conform a wide spectrum of diverse renal syndromes. Hypertension is one of the most frequent, but less commonly recognized renal alteration. It can be difficult to control as its origin is renovascular. Renal vascular thrombosis can be arterial or venous. Other alterations are renal infarction and vascular thrombosis in arterial territories. Venous thrombosis can be present in primary and secondary antiphospholipid syndrome; it presents with worsening of previous proteinuria or nephrotic syndrome, hypertension and renal failure. Antiphospholipid syndrome nephropathy is a vascular disease that affects glomerular tuft, interstitial vessels, and peritubular vessels; histopathology characterizes the renal lesions as acute or chronic, the classic finding is thrombotic microangiopathy, that leads to fibrosis, tubule thyroidization, focal cortical atrophy, and glomerular sclerosis. Antiphospholipid syndrome nephropathy can also complicate patients with systemic lupus erythematosus, and there is vast information supporting the worse renal prognosis in this group of patients with the classic histopathologic lesions. Treatment consists of anticoagulation, as for other thrombotic manifestations of antiphospholipid syndrome. There is some evidence of glomerulonephritis as an isolated lesion in patients with antiphospholipid syndrome. The most frequently reported glomerulonephritis is membranous; with some reports suggesting that immunosuppressive treatment may be effective. Patients with end stage renal disease commonly are positive for antiphospholipid antibodies, but it is not clear what is the role of aPL in this setting. Patients with vascular access may have complications in the presence of antibodies so that anticoagulation is recommended. Patients ongoing renal transplant with persistent antiphospholipid antibody positivity may have early and late graft failure.
抗磷脂综合征是一种复杂的自身免疫性疾病，其特征为血管血栓形成、产科、血液学、皮肤和心脏表现。在该疾病的最初描述中并未认识到抗磷脂综合征患者的肾脏疾病，但后来广泛研究了该综合征的肾脏表现。抗磷脂综合征的肾脏表现符合广泛的不同肾脏综合征谱。高血压是最常见的表现之一，但不太常见。由于其起源于肾血管，因此很难控制。肾血管血栓形成可以是动脉性或静脉性的。其他改变为肾梗死和动脉性血管血栓形成。静脉血栓形成可存在于原发性和继发性抗磷脂综合征中；其表现为蛋白尿或肾病综合征恶化、高血压和肾衰竭。抗磷脂综合征肾病是一种血管疾病，影响肾小球簇、间质血管和肾小管周围血管；组织病理学特征为急性或慢性病变，典型表现为血栓性微血管病，导致纤维化、肾小管甲状腺化、局灶性皮质萎缩和肾小球硬化。抗磷脂综合征肾病也可使系统性红斑狼疮患者复杂化，并且有大量信息支持该组患者的肾脏预后较差，其具有典型的组织病理学病变。治疗包括抗凝治疗，如抗磷脂综合征的其他血栓形成表现。有一些证据表明抗磷脂综合征患者存在孤立性肾小球肾炎。最常报道的肾小球肾炎为膜性，一些报告表明免疫抑制治疗可能有效。终末期肾病患者常为抗磷脂抗体阳性，但抗磷脂抗体在这种情况下的作用尚不清楚。存在抗体的血管通路患者可能有并发症，因此建议抗凝治疗。持续抗磷脂抗体阳性的正在进行肾移植的患者可能会出现早期和晚期移植物失功。
