Complete Remission of the Nephrotic Syndrome due to Focal Glomerular Sclerosis Achieved with Low Density Lipoprotein Adsorption Alone

Yokoyama, Keitaro; Sakai, Souichi; Yamaguchi, Yutaka; Suzuki, Yoshio; Hinoshita, Fumihiko; Hara, Sigeko; Yamada, Akira; Ogura, Yuichiro; Kawaguchi, Yoshindo; Sakai, Osamu

doi:10.1159/000188863

Cited by 15 publications

(16 citation statements)

References 5 publications

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“…Therefore, it is possible to suggest that rapid improvement of hyperlipidemia, including both high LDL and VLDL levels, by LDL-A may upregulate the steroid binding of systemic cells in NS. On the other hand, as LDL-A could induce the remission of NS before using steroid [22], the mechanism of action by LDL-A cannot be explained only by the improvement of steroid responsiveness.…”

Section: Discussionmentioning

confidence: 99%

Significantly Rapid Relief from Steroid-Resistant Nephrotic Syndrome by LDL Apheresis Compared with Steroid Monotherapy

Muso

Mune²,

Fujii

et al. 2001

Nephron

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Rapid amelioration of hypercholesterolemia by LDL apheresis (LDL-A) was performed for long-standing nephrotic syndrome (NS) with hyperlipidemia due to focal segmental glomerulosclerosis (FGS) and the clinical data and prognosis were compared between LDL-A-treated and nontreated groups. Seventeen steroid-resistant NS patients treated with LDL-A (LDL-A group) and 10 NS patients treated with steroids only (steroid-monotherapy (SM) group) were compared. Serum cholesterol and phospholipid levels were significantly lowered only in the LDL-A group (p < 0.01, respectively). The LDL-A group showed a significant decrease of urinary protein (UP, p < 0.01) and increase of serum albumin (p < 0.05). Average time needed to achieve a decrease of UP to less than nephrotic range (< 3.5 g/day) was significantly shorter in the LDL-A group than in the SM group (p < 0.01). Although this is not a prospective study, it is highly expected that a rapid improvement of hypercholesterolemia by LDL-A in steroid-resistant NS will provide more rapid relief from NS than steroid therapy alone.

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Section: Discussionmentioning

confidence: 99%

Significantly Rapid Relief from Steroid-Resistant Nephrotic Syndrome by LDL Apheresis Compared with Steroid Monotherapy

Muso

Mune²,

Fujii

et al. 2001

Nephron

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“…In addition, Yokoyama et al [31] found that LDL apheresis improved the response to steroid therapy in the patients of FSGS associated with nephrotic syndrome resistant to steroid, and that only electron microscopy was able to detect histological recovery in the patients who showed a decrease of proteinuria after LDL apheresis. They also reported a case with primary FSGS in whom a complete remission of nephrotic state was achieved with LDL apheresis alone [32]. In the only prospective study reported by Stenvinkel and Swedish group in 2000 [33], LDL apheresis caused a rapid 30-40% decrease in serum cholesterol and plasma Lp (a) levels in patients with nephrotic syndrome.…”

Section: ) Nephrotic Syndromementioning

confidence: 96%

Advances in apheresis therapy for glomerular diseases

et al. 2007

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This article overviewed the immunomodulation effects and clinical evidence of apheresis in renal diseases, especially primary and secondary glomerulonephritis. A considerable permeability factor(s) derived from circulating T cells is speculated to have a crucial role in proteinuria of nephrotic syndrome (NS). Plasma exchange (PE), immunoadsorption (IAPP) using Protein A sepharose cartridges, low density lipoprotein apheresis and lymphocyte apheresis (LCAP) were tried to remove such factors or pathogenic T cells. Other glomerular diseases induced by specific antibodies such as anti-glomerular basement membrane antibodies, anti-neutrophil cytoplasmic antibodies and immune-complexes were also treated with PE, double filtration plasma apheresis, IAPP and LCAP. The recommendations based on the evidence from recent randomized controlled studies have been established in apheresis therapy for the treatment of various glomerular diseases.

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“…Although the pathogenesis of FSGS is not clear, secondary hyperlipidemia is implicated as a possible factor in the renal dysfunction [3]. Recently, LDL-A has been proposed as a therapeutic intervention for patients with steroid-resistant FSGS [4][5][6][7][8]. Muso et al [7] reported that in adult patients with steroid-resistant NS due to FSGS or minimal change nephropathy, LDL-A ameliorated the severe proteinuria and improved renal function, along with the alleviation of hyperlipidemia, and achieved remission.…”

Section: Discussionmentioning

confidence: 99%

“…Although the pathogenesis of the disease has not been well elucidated, secondary hyperlipidemia plays a pivotal role in the progression of the renal injury [3]. Low-density lipoprotein apheresis (LDL-A) has been extended to the treatment of refractory NS due to steroid-resistant FSGS, minimal change nephropathy, membranous nephropathy, or diabetic nephropathy [4][5][6][7][8][9][10]. LDL-A improved abnormal lipid metabolism loss remained at 2-4 g·day −1 and Up/Ucr continued to show a value of 5-11 ( Fig.…”

Section: Introductionmentioning

confidence: 99%

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Low-density lipoprotein apheresis in a pediatric patient with refractory nephrotic syndrome due to focal segmental glomerulosclerosis

et al. 2009

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Focal segmental glomerulosclerosis (FSGS) often leads to refractory nephrotic syndrome (NS). A high level of low-density lipoprotein (LDL) is a risk factor for the progression of NS. An 8-year-old girl presented with severe proteinuria refractory to steroid therapy. She was diagnosed with non-IgA diffuse mesangial proliferative glomerulonephritis. Oral prednisolone, methylprednisolone (mPL) pulse therapy, and cyclosporine and cyclophosphamide therapy failed to achieve remission. Follow-up renal biopsy revealed FSGS. Her serum level of LDL was high, and LDL-apheresis (LDL-A) was performed five times, followed by mPL pulse therapy. Urinary protein decreased from 2-4 g x day(-) to 0.5-1.0 g x day(-). LDL-A may be beneficial in the treatment of multidrug-resistant FSGS.

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Complete Remission of the Nephrotic Syndrome due to Focal Glomerular Sclerosis Achieved with Low Density Lipoprotein Adsorption Alone

Cited by 15 publications

References 5 publications

Significantly Rapid Relief from Steroid-Resistant Nephrotic Syndrome by LDL Apheresis Compared with Steroid Monotherapy

Significantly Rapid Relief from Steroid-Resistant Nephrotic Syndrome by LDL Apheresis Compared with Steroid Monotherapy

Advances in apheresis therapy for glomerular diseases

Low-density lipoprotein apheresis in a pediatric patient with refractory nephrotic syndrome due to focal segmental glomerulosclerosis

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