Accelerated recovery from nephrotic syndrome with acute renal failure by double filtration plasmapheresis in a patient with lupus podocytopathy

Abstract

We describe a case of an adult female who presented with nephrotic syndrome. She was diagnosed with systemic lupus erythematosus with serum antinuclear antibodies, leucopenia with lymphopenia, butterfly erythema, and nephrotic syndrome. Renal biopsy revealed normal glomeruli with diffuse effacement of the foot processes, consistent with lupus podocytopathy. Although human albumin replacement was performed initially, acute renal failure developed rapidly. Therefore, she was treated with double filtration plasmapheresis (DFPP) in addition to oral steroid. After steroid therapy combined with DFPP, the renal function and proteinuria improved rapidly. Although the impact of DFPP on the treatment of lupus nephritis remains to be delineated, our observations suggest that DFPP in lupus podocytopathy played a pivotal role in facilitating the early recovery from renal injuries. Because of the rapid improvement of renal function without any change in body weight by DFPP, acute renal failure in the setting of lupus podocytopathy might contribute to an alternative pathophysiological factor for the diminished glomerular filtration rate, similar to that observed in the setting of idiopathic minimal change glomerulopathy.

Glucocorticoid with or without additional immunosuppressant therapy for patients with lupus podocytopathy: a retrospective single-center study

“…Additional immunosuppressive agents including CTX, 14 AZA, 9,14 MMF, 3,31,32 cyclosporine (CsA), 12,15 and anti-CD20 monoclonal antibody, 33 as well as double filtration plasmapheresis 23 were reported to be combined with glucocorticoid for inducing remission of lupus podocytopathy. However, all of these publications were case reports and most were indicated in patients who were refractory to glucocorticoid.…”

“…Most patients with lupus podocytopathy present with nephrotic syndrome, some patients also display acute kidney injury. [21][22][23] The treatment regimen for lupus podocytopathy reported in the literature is similar to that used for primary MCD or FSGS, with glucocorticoids being the most widely prescribed agents. Recently, clinical practice guidelines for lupus nephritis have recommended glucocorticoid monotherapy or glucocorticoid plus immunosuppressant therapy for the treatment of lupus podocytopathy with nephrotic syndrome.…”

“…Most patients with lupus podocytopathy present with nephrotic syndrome, some patients also display acute kidney injury. 21–23…”

Labile Heme Aggravates Renal Inflammation and Complement Activation After Ischemia Reperfusion Injury

“…In this study treatment with the heme scavenger human serum albumin (HSA) prior to IRI reduced pro-inflammatory cytokine release and C3aR expression in the renal tissue after prolonged IRI. HSA is broadly used in the clinic for the treatment of patients with ascites and chronic liver disease (42,43) and in the context of plasmapheresis in certain renal diseases (44). However, it is noteworthy that the serum protein hemopexin has a markedly higher binding affinity for heme than albumin.…”

Change of Chylous Ascites During Low‐Density Lipoprotein Apheresis in a Patient With Idiopathic Nephrotic Syndrome

“…A 32-year-old Japanese man presenting with rapid onset of generalized edema was diagnosed with minimal change nephrotic syndrome (MCNS) and was treated with intravenous methylprednisolone at a dose of 500 mg daily for 3 days followed by oral prednisolone (50 mg/day) in 2002. However, the clinical findings indicating steroid-resistance and high pro-teinuria selectivity index (0.29), led us to suspect focal segmental glomerulonephrosis (FSGS).Therefore, we also applied cyclosporin A (CyA) and therapeutic apheresis (low-density lipoprotein [LDL] apheresis: LDL absorption [LDL-A] using a dextran sulfate cellulose column, double filtration plasmapheresis [DFPP], as described previously) (2). After the initiation of CyA (100 mg/day) and LDL apheresis, the renal function and severe edema improved with no obvious improvement of the urinary protein excretion (UPE) or hypoalbuminemia.…”