Low-density lipoprotein apheresis in a patient with arteriosclerosis obliterans and light chain deposition disease.

Abstract

A 49-year-old women with arteriosclerosis obliterans (ASO) complicated with light chain deposition disease (LCDD) is described. Renal biopsy showed a diffuse mesangial nodular lesion and tubulointerstitial changes. Congo red and lambda light chain staining were negative; however, the kappa light chain was positive in both glomeruli and tubular basement membranes by immunostaining. Using electron microscopy, electron-dense materials were found within glomerular basement membrane, mesangium and tubular basement membrane. The patient had renal dysfunction and nephrotic syndrome with progressive skin ulcers in the left leg. The patient was diagnosed as ASO with LCDD. She received low-density lipoprotein (LDL) apheresis once weekly for 10 consecutive weeks. Serum total cholesterol and phospholipid levels were decreased, and serum creatinine and blood urea nitrogen levels also tended to decline after treatment. Urinary protein excretion was reduced markedly, and hypoalbuminemia was also improved. Ischemic symptoms including leg pain and leg coldness and numbness improved after apheresis. The walking distance increased on a treadmill. The skin temperature was increased from 33.8 degrees C to 35.5 degrees C after apheresis and the skin ulcers were also improved. Plasma nitric oxide (NO) levels were increased from 66.0 microM/l to 88.0 microM/l and plasma endothelin (ET)-1 levels were decreased from 14.5 pg/ml to 5.8 pg/ml after apheresis. LDL apheresis was effective in ameliorating hyperlipidemia, massive proteinuria, hypoalbuminemia and high serum creatinine levels in an LCDD patient with nephrotic syndrome. Furthermore, we showed beneficial effects of LDL apheresis on skin ulcers due to ischemia in an ASO patient complicated with LCDD.