Complete remission of steroid-resistant minimal-change nephrotic syndrome by cyclosporin after additional low-density lipoprotein apheresis treatment

Abstract

Steroid-resistant nephrotic syndrome (SRNS) is still difficult to treat in spite of the introduction of various immunosuppressive drugs. In recent years, low-density lipoprotein apheresis (LDL-A) has been used to treat SRNS, mainly on focal glomerular sclerosis (FGS), and some cases have shown amelioration of the nephrotic syndrome. We describe here a case of steroid-resistant minimal-change nephrotic syndrome (MCNS), which was also unresponsive to cyclosporin A (CsA), but attained remission after the additional LDL-A treatment. A 20-year-old man with MCNS was treated with prednisolone at the dose of 40 mg/day for 6 weeks, but his nephrosis continued. The administration of CsA (2.5mg/kg/day) induced remission temporarily for 2 months. However his nephrosis soon recurred, and persisted without improvement in spite of an increase in the CsA dose (4.5mg/kg/day). After 6 LDL-A treatments given concomitantly with the administration of CsA (5mg/kg/day), the urinary protein excretion remarkably reduced, and the patient remained free of the disease. Although the precise mechanism of hyperlipidemia on CsA is still unknown, it can be speculated that the rapid improvement of hyperlipidemia by LDL-A might strengthen the effect of CsA, presumably through the increased cellular uptake of CsA. LDL-A might be useful for ameliorating steroid resistant MCNS, which also is unresponsive to immunosuppressive drugs, such as CsA.