Low-density Lipoprotein Receptor Activities, Lipids, Apolipoprotein, and Clinical Course of Patients with Steroid-resistant Nephrotic Syndrome Treated with Low-density Lipoprotein Apheresis: A Case Series

• Published in: Internal Medicine Vol. 63; no. 3; pp. 433 - 438
• Main Authors: Shima, Hisato, Higashiguchi, Yusuke, Doi, Toshio, Harada, Megumi, Okamoto, Takuya, Inoue, Tomoko, Tashiro, Manabu, Okada, Kazuyoshi, Minakuchi, Jun
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01.02.2024; Japan Science and Technology Agency
• Subjects: Apheresis; Apolipoproteins; Apolipoproteins - therapeutic use; Blood Component Removal; Case Report; Cholesterol; Cyclosporine - therapeutic use; Cyclosporins; Disease Progression; focal segmental glomerulosclerosis; Glomerulosclerosis, Focal Segmental; High density lipoprotein; Humans; hyperlipidemia; LDL receptor activity; Lipids; Lipoproteins, LDL - therapeutic use; Low density lipoprotein; Low density lipoprotein receptors; low-density lipoprotein apheresis; Nephrotic syndrome; Nephrotic Syndrome - drug therapy; Receptor density; Receptors, LDL; Steroid hormones; Triglycerides; low-density lipoprotein apheresis; hyperlipidemia; LDL receptor activity; focal segmental glomerulosclerosis; nephrotic syndrome
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.1922-23

We herein report three cases of steroid-resistant nephrotic syndrome successfully treated with low-density lipoprotein apheresis (LDL-A). All patients were treated with a combination of steroids, cyclosporine, and LDL-A. In all cases, the serum concentrations of LDL, total and high-density lipoprotein cholesterol, and triglycerides were significantly lowered following LDL-A administration. Furthermore, the estimated LDL receptor activity increased, while both serum LDL and total cholesterol levels decreased, suggesting that LDL-A increases LDL receptor activity by driving changes in serum cholesterol concentration. This case series suggests that LDL-A increases LDL receptor activity, which may improve the intracellular uptake of cyclosporine.