妊娠合并单克隆IgG沉积的增生性肾小球肾炎伴大新月体形成1例

A case of proliferative glomerulonephritis with large crescent formation in pregnancy with monoclonal IgG deposition

患者青年女性,有梅毒病史,肉眼血尿起病,慢性病程,孕中后期病情加重,临床表现为肉眼血尿、肾病综合征、急进性肾小球肾炎需透析,补体C3、C4下降,血、尿免疫固定电泳均可见单克隆IgG-Kappa沉积。肾脏病理光镜下为膜增生性肾小球肾炎伴新月体形成,免疫荧光IgG亚型中只有单一IgG3沉积,轻链Kappa呈限制性表达,诊断为伴单克隆IgG(IgG3-Kappa型)沉积的增生性肾小球肾炎(proliferative glomerulonephritis with monoclonal IgG deposits,PGNMID)。该病例为兼具梅毒病史和妊娠期间病情加重的首例报道,采用硼替佐米+环磷酰胺+地塞米松化疗方案,最终患者摆脱透析,肾功能恢复,蛋白尿好转。

The patient was a young woman with a history of syphilis,gross hematuria onset,chronic course,exacerbation in the second and third trimesters.The clinical manifestations were gross hematuria,nephrotic syndrome,rapidly progressive glomerulonephritis requiring dialysis,decreased complement C3 and C4,and monoclonal IgG-kappa deposition in blood and urine immunofixation electrophoresis.Membranous proliferative glomerulonephritis with crescent formation under renal pathological light microscopy,only single IgG3 deposition in the immunofluorescent IgG subtype,and restrictive expression of light chain kappa,were diagnosed as proliferative glomerulonephritis with monoclonal IgG(IgG3-kappa)deposits(PGNMID).The was the first case with a history of syphilis that worsened during pregnancy.After treatment with bortezomib,cyclophosphamide,and dexamethasone,the patient was finally released from dialysis,renal function recovered,and proteinuria improved.