Túri S, Haszon I, Bodrogi T
Department of Paediatrics, University Medical School of Szeged, Hungary.
Acta Paediatr Hung. 1988;29(3-4):293-8.
A case is reported of systemic lupus erythematosus (SLE) associated with steroid-resistant nephrotic syndrome. Serial plasma exchanges (PE) combined with prednisolone treatment induced complete normalization of the immunological findings: the anti-DNA antibody, antinuclear antibody, LE cell phenomenon and circulatory immune complexes became negative. The prostacyclin (PGI2) production-supporting activity in the plasma increased to the control range; inhibitors of PGI2 production were eliminated. The creatinine clearance normalized, the urinary protein excretion decreased significantly, and the facial erythema disappeared. Continued treatment with chlorambucil + low-dose prednisolone led to a complete and stable remission of the nephrotic syndrome, and the C3 complement normalized. The low level of PGI2 production-supporting activity in the plasma may be explained by the inhibitor of PGI2 production. PE + immunosuppressive therapy might have beneficial effects on the immunological changes and PGI2 metabolism, and also on the remission of SLE-nephrotic syndrome.
报告了一例系统性红斑狼疮（SLE）合并类固醇抵抗性肾病综合征的病例。连续血浆置换（PE）联合泼尼松龙治疗使免疫学指标完全恢复正常：抗DNA抗体、抗核抗体、LE细胞现象及循环免疫复合物均转阴。血浆中前列环素（PGI2）生成支持活性升至对照范围；PGI2生成抑制剂被清除。肌酐清除率恢复正常，尿蛋白排泄显著减少，面部红斑消失。苯丁酸氮芥+小剂量泼尼松龙持续治疗使肾病综合征完全缓解且病情稳定，C3补体恢复正常。血浆中PGI2生成支持活性水平低可能是由PGI2生成抑制剂所致。PE+免疫抑制治疗可能对免疫学改变和PGI2代谢有益，对SLE肾病综合征的缓解也有益。
