Abstract：
Systemic lupus erythematosus (SLE) patients clinically presenting with nephrotic syndrome demonstrating minimal change disease (MCD), mesangial proliferation (MsP) or focal segmental glomerulosclerosis (FSGS), while on electronic microscopy, diffuse podocyte foot process effacement in absence of sub-epithelial or sub-endothelial deposition is the only morphological feature and now diagnosed as lupus podocytopathy. Lupus podocytopathy with glomerular morphology of MCD or MsP usually presents with typical nephrotic syndrome and sensitive to glucocorticoid treatment, but the relapse rate could reach up to 90% on maintenance treatment with glucocorticoid alone. Glucocorticoid plus other immunosuppressive agents could significantly decrease the relapse rate. Lupus podocytopathy with FSGS presents with a higher rate of acute kidney injury and less sensitivity to glucocorticoid treatment. The long-term outcomes of lupus podocytopathy are optimistic, but pathological transition could occur after renal relapses. The unique clinical and morphological features of lupus podocytopathy indicate that this special SLE-associated glomerulopathy should be included in the upcoming revised pathological classification of lupus nephritis.
摘要：
系统性红斑狼疮（SLE）患者临床表现为肾病综合征，表现为微小病变（MCD），系膜增生（MsP）或局灶节段肾小球硬化（FSGS），而在电子显微镜上，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是唯一的形态学特征，现在被诊断为狼疮足细胞病。合并MCD或MsP肾小球形态的狼疮足细胞病通常表现为典型的肾病综合征，对糖皮质激素治疗敏感。但单用糖皮质激素维持治疗的复发率可达90%。糖皮质激素联合其他免疫抑制剂可显著降低复发率。FSGS的狼疮足细胞病具有较高的急性肾损伤发生率和对糖皮质激素治疗的敏感性。狼疮足细胞病的长期结果是乐观的，但是肾脏复发后可能发生病理转变。狼疮足细胞病的独特临床和形态学特征表明，这种特殊的SLE相关性肾小球病应该包括在即将修订的狼疮肾炎病理分类中。
