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系统性红斑狼疮患者临床表现为肾病综合征,肾活检病理表现为肾小球轻微病变(MCD)、系膜增生(Ms P)或局灶节段性肾小球硬化(FSGS),超微结构以足细胞足突广泛融合为特征,毛细血管袢内皮下及上皮侧无免疫沉积物的肾损害称为狼疮足细胞病。肾小球病理改变为MCD和Ms P的狼疮足细胞病患者,血尿发生率低,激素治疗敏感,但单用激素维持的复发率高达90%,激素联合其他免疫抑制剂维持可显著降低复发率。而病理表现为FSGS的狼疮足细胞病患者,急性肾损伤的发生率高,肾小管间质损伤重,激素治疗缓解率低。狼疮足细胞病复发后可发生病理转型,远期预后良好。狼疮足细胞病应作为一类特殊类型狼疮性肾炎纳入新的病理分型体系中。
The clinical manifestations of systemic lupus erythematosus patients with nephrotic syndrome, renal biopsy pathology showed mild glomerular lesions (MCD), mesangial hyperplasia (Ms P) or focal segmental glomerulosclerosis (FSGS), ultrastructure The podocyte foot process is characterized by a wide range of fusion, renal capillary lesion and subcutaneous epithelial side of the immune deposits known as lupus psoas disease. Patients with lupus podocytopathy with pathological changes of glomerular pathology were MCD and Ms P, with low incidence of hematuria and hormone therapy. However, the recurrence rate was as high as 90% with hormone alone. Hormone and other immunosuppressive agents could significantly reduce the relapse rate . The pathological presentation of FSGS in patients with lupus podocytic disease, the incidence of acute renal injury, tubulointerstitial injury, hormone therapy, low response rate. Pathological transformation of lupus psoas disease may occur after the recurrence, the long-term prognosis is good. Lupus psoons disease should be included as a new type of pathological typing system for a particular type of lupus nephritis.