Low density lipoprotein apheresis therapy for steroid-resistant nephrotic syndrome

Abstract

Low density lipoprotein apheresis therapy for steroid-resistantFocal and segmental glomerulosclerosis (FGS) is a nephrotic syndrome.disease manifesting severe nephrotic syndrome (NS) that Background. The pathogenic role of hyperlipidemia in longthe latter months of 1989 we performed a random protocol LDL-A therapy that included nine episodes of steroid-resistant NS in eight patients who had been diag-Key words: thromboxane B 2 , progressive renal disease, tubulointerstinosed with FGS or MCNS [6]. For the multicenter study tial injury, focal segmental glomerulosclerosis, minimal change nephrotic syndrome.with a fixed protocol, 17 steroid-resistant NS patients (14 FGS and 3 MCNS) were entered into the study.

Clinical practice guideline for pediatric idiopathic nephrotic syndrome 2013: medical therapy

“…However, some clinical studies in adults show benefits of LDL apheresis in inducing remission of steroid-resistant nephrotic syndrome [142][143][144][145]. In children, a clinical study involving 11 patients with refractory steroid-resistant nephrotic syndrome reported a (complete or partial) remission rate of 63 %, suggesting that LDL apheresis can help induce remission of refractory steroid-resistant nephrotic syndrome in children [146].…”

“…Specific mechanism(s) behind the beneficial effects of LDL apheresis on this disease remain unclear, but it has been suggested to go beyond the improvement of dyslipidemia. Some reports indicate that LDL apheresis can improve the responsiveness to steroids [143,144,146,147] and cyclosporine [148], and thus combination therapy of LDL apheresis with these drugs is recommended rather than LDL apheresis alone. Efficacy of LDL apheresis has been described to be associated with highly-selective proteinuria (one study showed that patients with complete remission after LDL apheresis had a lower selectivity index [SI], which is calculated as [urinary immunoglobulin G (IgG)/serum IgG] 9 [serum transferrin (Tf)/urinary Tf], of 0.05 ± 0.02, compared with 0.25 ± 0.04 in patients who did not respond to LDL apheresis [146]) or minor renal tubular interstitial damage [146], and thus early LDL apheresis after the onset of the disease is considered advisable [142,146].…”

“…Vascular access infection has also been reported [146]. Angiotensin-converting enzyme inhibitor therapy should be discontinued before LDL apheresis because of the possible shock with LDL apheresis during angiotensinconverting enzyme inhibitor therapy, but there have been no reports of serious side effects of LDL apheresis in patients with refractory steroid-resistant nephrotic syndrome [142][143][144][145][146][147]. However, since LDL apheresis requires a large amount of blood to fill the circuit, it is safe to limit the use of LDL apheresis to children weighing more than 30 kg.…”

Dyslipidemia in Chronic Kidney Disease: An Approach to Pathogenesis and Treatment

“…It is noteworthy that the treatment of nephrotic dyslipidemia with the application of LDL-apheresis [80, 81] or with the use of medications such as statins [82] and direct ACAT inhibitors [83] has a favorable effect on some NS-related parameters such as hypoalbuminemia and albuminuria. These observations clearly demonstrate the existence of a vicious circle involving proteinuria and dyslipidemia.…”

Minimal Change Nephrotic Syndrome after Allogenic Hematopoietic Stem Cell Transplantation

“…LDL-apheresis might absorb glomerular permeability factors (e.g. TNF-, IFN-) in addition to LDL and help decrease proteinuria in patients with steroid-resistant nephrotic syndrome (10). Our patient also underwent therapy with LDL-apheresis, but nephrotic syndrome did not go into remission during the short term.…”