Clinical characteristics of systemic lupus erythematosus complicated with thrombotic microangiopaopathy and therapeutic effect of induction-remission in children
AIM To analyze the clinical characteristics and therapeutic efficacy of systemic lupus erythematosus(SLE)complicated with thrombotic microangiopathy(TMA)in children.METHODS A retrospective analysis was conducted on the clinical data of 5 children with SLE and TMA in our hospital from January 2019 to January 2023.RESULTS The duration of SLE was(3.2±4.4)months,the age was(11.2±2.8)years,and the SLE disease activity index was severe SLE activity at the time of TMA diagnosis.There were 4 cases with hemolytic uremic syndrome,2 cases with malignant hypertension and 1 case with thrombotic thrombocytopenic purpura.All children were treated with rituximab,intravenous immunoglobulin(IVIG),methylprednisolone shock and cyclophosphamide shock after TMA.Four patients received continuous renal replacement therapy,3 patients received plasma exchange,and 1 child received peritoneal dialysis for six months before hemodialysis.1 of the 5 patients developed chronic renal insufficiency and received long-term hemodialysis treatment.Urine protein turned negative in 24h in 2 patients,renal function was normal,complete remission.The serum creatinine and creatinine clearance of 2 cases were normal after 2 months follow-up and partially relieved.CONCLUSION Children with SLE combined with TMA mostly occur in the severe active stage of the disease,and renal failure is more prominent.Rituximab combined with steriod shock and IVIG therapy can improve TMA to some extent.
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