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Low-density lipoprotein apheresis in a patient with arteriosclerosis obliterans and light chain deposition disease
T Nakamura1, Y Kawagoe, T Matsuda
1Department of Medicine, Shinmatsudo Central General Hospital, Chiba, Japan.
Insights
Low-density lipoprotein (LDL) apheresis improved kidney function, proteinuria, and skin ulcers in a patient with arteriosclerosis obliterans (ASO) and light chain deposition disease (LCDD). This treatment also enhanced circulation and reduced inflammatory markers.
Area of Science:
- Nephrology
- Cardiology
- Immunology
Background:
- Light chain deposition disease (LCDD) is a rare plasma cell dyscrasia characterized by the deposition of monoclonal immunoglobulin light chains in various organs, primarily the kidneys.
- Arteriosclerosis obliterans (ASO) is a form of peripheral artery disease (PAD) caused by atherosclerosis, leading to reduced blood flow to the extremities.
Observation:
- A 49-year-old female patient presented with renal dysfunction, nephrotic syndrome, and progressive skin ulcers on her left leg, secondary to arteriosclerosis obliterans (ASO) complicated with light chain deposition disease (LCDD).
- Renal biopsy revealed diffuse mesangial nodular lesions and tubulointerstitial changes, with positive kappa light chain staining in glomeruli and tubular basement membranes.
- Electron microscopy confirmed electron-dense deposits in the glomerular basement membrane, mesangium, and tubular basement membrane.
Findings:
- Treatment with low-density lipoprotein (LDL) apheresis for 10 weeks led to significant improvements in serum lipid profiles, renal function markers (serum creatinine, blood urea nitrogen), and urinary protein excretion.
- The patient experienced marked reduction in proteinuria, improvement in hypoalbuminemia, and amelioration of ischemic symptoms, including leg pain, coldness, and numbness.
- Skin ulcers showed improvement, with increased skin temperature and enhanced walking distance, alongside favorable changes in plasma nitric oxide (NO) and endothelin (ET)-1 levels.
Implications:
- LDL apheresis demonstrates therapeutic efficacy in managing hyperlipidemia, nephrotic syndrome, and renal dysfunction associated with LCDD.
- This case highlights the potential of LDL apheresis in improving ischemic skin manifestations in patients with coexisting ASO and LCDD.
- The study suggests that LDL apheresis may offer a beneficial treatment option for complex cases involving both renal and vascular complications.
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.
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