[Excellent antiproteinuric effect by LDL apheresis in a case with severe renal dysfunction due to focal segmental...

We report here a 19-year-old man with intractable nephrotic syndrome due to focal glomerulosclerosis (FGS) treated by low-density lipoprotein apheresis (LDL-A). The patient had been receiving several drugs, including steroids, cyclophosphamide, mizoribine and deoxysparguarine, for the past ten years, but the nephrotic syndrome was resistant to these drugs. Although the initial renal biopsy specimen showed minimal change-type lesions, the second biopsy specimen obtained 6 years later revealed typical FGS findings accompanied by lipid deposition (apoB) and macrophage infiltration (CD68) in the involved area. LDL-apheresis was performed ten times per course using a dextran sulfate cellulose column (Liposorba LA-15) as the LDL absorber and polysulfone hollow-fibers (Sulflux) as the plasma separator, processing a total of 3,000 ml of plasma during each apheresis. After treatment the serum levels of LDL and total cholesterol decreased to 50% and 58% of their initial levels, respectively. Immediately after the first course of treatment, the renal dysfunction did not improve, but a decrease in urinary protein was observed (from 43.7 g/day to 8 g/day). Two months later, because urinary protein increased and renal function decreased (Ccr 7 ml/min), a second course of treatment was started. However, his renal dysfunction did not improve and urinary protein did not decrease. In conclusion, in FGS with-progressive renal failure, renal histological findings of positive APO-B, CD68 (macrophage) in sclerotic lesion may be indications of effective LDL-apheresis.