Abstract
We report a case of systemic lupus erythematosus (SLE) associated with hemolytic uremic syndrome (HUS). The patient was a 13-year-old boy who had complained of nausea and diarrhea. Abnormal urinalysis, pancytopenia and renal dysfunction were revealed. The immunological studies showed an elevation of antinuclear antibody and anti-double-stranded DNA antibody titers with a low complement level. Renal biopsy specimens showed diffuse membranous glomerulonephritis with microthrombi in the glomerular capillary lumen. He was diagnosed as having SLE with HUS. Methylprednisolone pulse therapy was performed. Renal function, proteinuria and hematuria were gradually improved. Prednisolone and an immunosuppressive agent (mizoribine) were prescribed per os. In our case, diarrheal prodrome was present, so gastro-enteritis was suggested as the trigger of HUS, but the causal agent was not detected. HUS was considered to be an accelerator in the renal lesions of SLE. There have been few reported cases in children of SLE associated with HUS.