難治性ネフローゼ症候群とLDLアフェレシス(<特集>腎疾患とアフェレシス-最近の知見-)

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  • 武曾,恵理
    財団法人田附興風会医学研究所北野病院腎臓内科

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  • Low-Density Lipoprotein Apheresis in Refractory Nephrotic Syndrome

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Since 1980, Lipid nephrotoxicity has been pointed out as one of the risk factors not only for deterioration of renal function but systemic cardiovascular events. In refractory nephrotic syndrome (NS), in addition to the direct aggravating effect of heavy proteinuria on longstanding hypercholesterolemia is known to provide not only poor renal survival but life survival due to life-threatening cardiovascular disease (CVD). Low-density lipoprotein apheresis (LDL-A) using a dextran sulfate cellulose column in combination with conventional drug treatment is known to yield rapid relief of NS, and improve drug sensitivity especially in drug-resistant focal segmental glomerulosclerosis (FGS). As for the mechanisms of the beneficial effects of LDL-A, lowering oxidized LDL may down-regulate proliferation of mesangial cells and normalize the monocyte and macrophage function suppressing up-regulated production of inflammatory cytokines. Furthermore, aggressive lowering of LDL increases susceptibility to steroid and Cyclospone A (CsA). In Japan, several retrospective data concerning the beneficial effect of LDL-A on refractory NS, mainly of FGS, have been accumulated. National survey of the report of these cases have revealed that LDL-A could provide the relief from refractory NS in almost 60%. For further trail to establish the convincing evidence of these effect nationwide prospective cohort study (POLARIS) is now undergoing. Preliminary analysis have suggested the remission rate from NS is more than 50% not only in FGS but in any type of NS diseases. The widespread usage of LDL-Apheresis in refractory NS cases should be recommended.

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