狼疮足细胞病是系统性红斑狼疮（SLE）的肾小球病变，其特征是弥漫性足细胞足突清除（FPE），无免疫复合物（IC）沉积或仅有系膜IC沉积。在患有SLE的儿童中很少见。一名13岁女孩符合2019年欧洲抗风湿病联盟（EULAR）/美国风湿病学会（ACR）SLE分类标准，基于ANA阳性;面部皮疹;血小板减少;蛋白尿;和抗磷脂（aPL）抗体阳性，包括狼疮抗凝药（LAC），抗β2糖蛋白-I抗体（抗β2GPI），和抗心磷脂抗体（aCL）。肾脏病变以3+蛋白尿为特征，4.2毫克/毫克斑点（随机）尿蛋白肌酐比，和疾病开始时的低蛋白血症（26.2g/l）。肾活检结果显示免疫球蛋白A（IgA）免疫荧光（IF）阴性，IgM,纤维蛋白原（Fb），C3和C1q,除了微弱的IgG；在光学显微镜下正常的肾小球外观；和通过电子显微镜（EM）在没有上皮下或内皮下沉积的情况下弥漫性足细胞足突消失（FPE）。耳廓表皮和真皮的组织病理学显示小血管中透明血栓。患者符合基于微血管血栓形成和持续阳性aPL抗体的APS分类标准。她对糖皮质激素和免疫抑制剂的组合有反应。我们的研究加强了考虑LP和APS潜在共存的必要性。临床医生应该意识到APS的潜在存在，在诊断为具有NS的LP和aPL抗体阳性的患者中，特别是三重aPL抗体(LCA，抗β2GPI，和aCL）。
Lupus podocytopathy is a glomerular lesion in systemic lupus erythematosus (SLE) characterized by diffuse podocyte foot process effacement (FPE) without immune complex (IC) deposition or with only mesangial IC deposition. It is rarely seen in children with SLE. A 13-year-old girl met the 2019 European League Against Rheumatism (EULAR)/ American College of Rheumatology (ACR) Classification Criteria for SLE based on positive ANA; facial rash; thrombocytopenia; proteinuria; and positive antiphospholipid (aPL) antibodies, including lupus anticoagulant (LAC), anti-β2 glycoprotein-I antibody (anti-β2GPI), and anti-cardiolipin antibody (aCL). The renal lesion was characterized by 3+ proteinuria, a 4.2 mg/mg spot (random) urine protein to creatinine ratio, and hypoalbuminemia (26.2 g/l) at the beginning of the disease. Kidney biopsy findings displayed negative immunofluorescence (IF) for immunoglobulin A (IgA), IgM, fibrinogen (Fb), C3, and C1q, except faint IgG; a normal glomerular appearance under a light microscope; and diffuse podocyte foot process effacement (FPE) in the absence of subepithelial or subendothelial deposition by electron microscopy (EM). Histopathology of the epidermis and dermis of the pinna revealed a hyaline thrombus in small vessels. The patient met the APS classification criteria based on microvascular thrombogenesis and persistently positive aPL antibodies. She responded to a combination of glucocorticoids and immunosuppressive agents. Our study reinforces the need to consider the potential cooccurrence of LP and APS. Clinicians should be aware of the potential presence of APS in patients with a diagnosis of LP presenting with NS and positivity for aPL antibodies, especially triple aPL antibodies (LCA, anti-β2GPI, and aCL).
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