A Prospective Observational Survey on the Long-Term Effect of LDL Apheresis on Drug-Resistant Nephrotic Syndrome

Muso, Eri; Mune, Masatoshi; Hirano, Tsutomu; Hattori, Motoshi; Kimura, Kenjiro; Watanabe, Tsuyoshi; Yokoyama, Hitoshi; Sato, Hiroshi; Uchida, Shunya; Wada, Takashi; Shoji, Tetsuo; Takemura, Tsukasa; Ogahara, Satoru; Sugiyama, Satoshi; Iino, Yuichi; Sakai, Soichi; Ogura, Y; Yukawa, Susumu; Nishizawà, Yoshiki; Yorioka, Noriaki; Imai, Enyu; Matsuo, Seiichi; Saito, Takao

doi:10.1159/000437338

Cited by 41 publications

(51 citation statements)

References 26 publications

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“…Initial results demonstrated nearly 50% reductions in both total cholesterol and LDL cholesterol levels during treatment 143 . A follow-up paper 144 demonstrated complete remission in 25% of the 44 patients enrolled in the study, and reported that an additional 23% of patients had partial remission (defined as <1 g of urinary protein per day). Furthermore, a case report demonstrated the induction of remission by lipid apheresis in an adult with rituximab-resistant nephrotic syndrome 145 .…”

Section: Treatment Of Dyslipidaemiamentioning

confidence: 99%

Dyslipidaemia in nephrotic syndrome: mechanisms and treatment

et al. 2017

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Nephrotic syndrome is a highly prevalent disease that is associated with high morbidity despite notable advances in its treatment. Many of the complications of nephrotic syndrome, including the increased risk of atherosclerosis and thromboembolism, can be linked to dysregulated lipid metabolism and dyslipidaemia. These abnormalities include elevated plasma levels of cholesterol, triglycerides and the apolipoprotein B containing lipoproteins VLDL and IDL; decreased lipoprotein lipase activity in the endothelium, muscle and adipose tissues; decreased hepatic lipase activity; and increased levels of the enzyme PCSK9. In addition, there is an increase in the plasma levels of immature HDL particles and reduced cholesterol efflux. Studies from the past few years have markedly improved our understanding of the molecular pathogenesis of nephrotic syndrome associated dyslipidaemia, and also heightened our awareness of the associated exacerbated risks of cardiovascular complications, progressive kidney disease and thromboembolism. Despite the absence of clear guidelines regarding treatment, various strategies are being increasingly utilized, including statins, bile acid sequestrants, fibrates, nicotinic acid and ezetimibe, as well as lipid apheresis, which seem to also induce partial or complete clinical remission of nephrotic syndrome in a substantial percentage of patients. Future potential treatments will likely also include inhibition of PCSK9 using recently developed anti PCSK9 monoclonal antibodies and small inhibitory RNAs, as well as targeting newly identified molecular regulators of lipid metabolism that are dysregulated in nephrotic syndrome.

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Section: Treatment Of Dyslipidaemiamentioning

confidence: 99%

Dyslipidaemia in nephrotic syndrome: mechanisms and treatment

et al. 2017

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“…A recent single arm observational study (POLARIS; N = 44) found that low‐density lipoprotein apheresis (LDL‐A) was able to ameliorate nephrotic syndrome in half of patients who were found to be drug resistant. Those patients who experienced disease remission continued to have a favorable outcome after 2 years of follow up . In this study, decreased urinary protein levels after treatment was most significantly correlated with a favorable 2‐year outcome.…”

Section: Introductionmentioning

confidence: 50%

Treatment‐resistant PLA2R‐negative membranous nephropathy responsive to low‐density lipoprotein apheresis

Szymanski

Waldman

Conry‐Cantilena

et al. 2018

J of Clinical Apheresis

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Idiopathic membranous nephropathy is the most common cause of nephrotic syndrome in nondiabetic adults. The antibody most often implicated is the M‐type phospholipase A2 receptor (PLA2R) antibody, found in >70% of primary membranous nephropathy cases. First‐line therapy is immunosuppressive in nature, but for patients who are treatment‐resistant there is a significant risk of end‐stage renal disease and mortality. Hypercholesterolemia is not only a side effect of nephrotic syndrome, but also its presence may worsen renal function. A recent single‐arm observational study in Japan found that low‐density lipoprotein apheresis (LDL‐A) was able to ameliorate nephrotic syndrome in half of patients who were resistant to medication. We present a case of treatment resistant PLA2R negative membranous nephropathy who had significant improvement following two courses of LDL‐A. To our knowledge, this is the first such reported case in the United States.

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“…In addition, the POLARIS study revealed that the change in the urinary protein after LDL-A may be a significant predictor of the outcome (2). …”

Section: Discussionmentioning

confidence: 99%

Long-term Low-density Lipoprotein Apheresis in a Patient with Refractory Idiopathic Membranous Glomerulonephritis

et al. 2017

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A 61-year-old Japanese man developed nephrotic syndrome (NS) due to idiopathic membranous glomerulonephritis (MGN). He received immunosuppressive therapy for two years, including prednisolone, cyclophosphamide, and cyclosporine A, but the NS persisted. Low-density lipoprotein apheresis (LDL-A) was initiated at a frequency of twice a month and continued for 9 years (203 sessions in total). His proteinuria reduced to less than 1 g daily after 9 years. LDL-A was stopped, and the NS has not relapsed for five years. This case suggests that long-term LDL-A therapy may be a treatment option for idiopathic MGN refractory to immunosuppressive therapy or short-term LDL-A.

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A Prospective Observational Survey on the Long-Term Effect of LDL Apheresis on Drug-Resistant Nephrotic Syndrome

Cited by 41 publications

References 26 publications

Dyslipidaemia in nephrotic syndrome: mechanisms and treatment

Dyslipidaemia in nephrotic syndrome: mechanisms and treatment

Treatment‐resistant PLA2R‐negative membranous nephropathy responsive to low‐density lipoprotein apheresis

Long-term Low-density Lipoprotein Apheresis in a Patient with Refractory Idiopathic Membranous Glomerulonephritis

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