Complete remission of intractable nephrotic syndrome by the treatment with a combination of low-density lipoprotein apheresis and steroids. —a case of report—

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  • ＬＤＬ吸着療法が著効した難治性ネフローゼ症候群の１例

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Abstract

  Focal segmental glomerulosclerosis (FSGS) is steroid-resistant, and not sensitive to various immunosuppressive drugs as well. We report here a 12-year-old boy with drug-resistant nephrotic syndrome. He was treated with prednisolone (PSL) at the dosage of 2mg/kg/day at first, but his nephrosis continued. Then we tried methyl-prednisolone pulse therapy and many other drugs including cyclosporin (CyA), mizoribine, HMG-CoA reductase inhibitor, but the nephrotic syndrome was resistant to these drug therapies. Although the administration of CyA in addition to steroids reduced proteinuria temporarily, we forced to reduce the dosage of CyA, for leukopenia, a rare side effect of CyA, occurred. So we tried the treatment of LDL apheresis (LDL-A) combined with the drug therapy. It is well accepted that the administration of CyA and/or steroids during or immediately after LDL-A treatment can take a better effect due to the remission of hyperlipidemia by LDL-A. However, in this case, we tried to restart the administration of steroid two months after LDL-A therapy, the proteinuria became sensitive to steroid therapy. Since serum lipid increased again at that time point, we therefore speculated that it is not the remission of hyperlipidemia which leads to the effect of steroid sensitivity after LDL-A therapy. Furthermore, no relapse has been observed during a follow-up period of 3-month till now. Although the precise mechanism of LDL-A is still uncovered, it can be conjectured that apheresis of unknown substances except cholesterol or the down-regulation of hyperlipidemia might improve steroid sensitivity. In conclusion, LDL-A might be useful for ameliorating drug-resistant nephrotic syndrome.

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