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.