Li Guo-Min, Li Yi-Fan, Zeng Qiao-Qian, Zhang Xiao-Mei, Liu Hai-Mei, Feng Jia-Yan, Shi Yu, Wu Bing-Bing, Xu Hong, Sun Li
National Children's Medical Center, Shanghai, China.
Department of Rheumatology, Children's Hospital of Fudan University, Shanghai, China.
Front Pediatr. 2022 Aug 19;10:950576. doi: 10.3389/fped.2022.950576. eCollection 2022.
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).
狼疮性足细胞病是系统性红斑狼疮（SLE）中的一种肾小球病变，其特征为弥漫性足细胞足突消失（FPE），无免疫复合物（IC）沉积或仅伴有系膜IC沉积。在儿童SLE患者中较为罕见。一名13岁女孩基于抗核抗体（ANA）阳性、面部皮疹、血小板减少、蛋白尿以及抗磷脂（aPL）抗体阳性（包括狼疮抗凝物（LAC）、抗β2糖蛋白-I抗体（抗β2GPI）和抗心磷脂抗体（aCL））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。疾病初期，肾脏病变表现为3+蛋白尿、随机尿蛋白肌酐比值为4.2mg/mg、低白蛋白血症（26.2g/l）。肾活检结果显示，免疫球蛋白A（IgA）、IgM、纤维蛋白原（Fb）、C3和C1q免疫荧光（IF）阴性，仅IgG弱阳性；光镜下肾小球外观正常；电镜（EM）显示弥漫性足细胞足突消失（FPE），无上皮下或内皮下沉积。耳廓表皮和真皮组织病理学检查显示小血管中有透明血栓。该患者基于微血管血栓形成和持续阳性的aPL抗体符合抗磷脂综合征（APS）分类标准。她对糖皮质激素和免疫抑制剂联合治疗有反应。我们的研究强调了需要考虑狼疮性足细胞病（LP）和抗磷脂综合征（APS）可能同时存在。临床医生应意识到，对于诊断为LP且表现为肾病综合征（NS）以及aPL抗体阳性（尤其是三联aPL抗体（LCA、抗β2GPI和aCL））的患者，可能存在APS。
