Remission of proteinuria in multidrug‐resistant idiopathic nephrotic syndrome following immunoglobulin immunoadsorption

Nattes, Elodie; Karaa, Danièle; Dehoux, Laurène; Kwon, Thérèsa; Deschênes, Georges

doi:10.1111/apa.14582

Cited by 3 publications

(4 citation statements)

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“… 15 A recent retrospective study by Nattes et al. 16 evaluated the efficacy of plasma protein (IA) in 14 children with refractory INS. This study reported a complete and partial remission rate of 64%, but all patients displayed relapse when IA was stopped.…”

Section: Discussionmentioning

confidence: 99%

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Apheresis in Adult With Refractory Idiopathic Nephrotic Syndrome on Native Kidneys

Moret

Ganea

Dao

et al. 2021

Kidney International Reports

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Background Apheresis is the gold standard for idiopathic nephrotic syndrome (INS) relapse after transplantation, but it remains unknown whether such treatment is useful for adults with refractory INS on native kidneys. Methods This retrospective study included patients older than 16 years with biopsy-proven refractory (persistent nephrotic syndrome on corticosteroids plus at least 1 immunosuppressive drug) INS treated by apheresis and followed for at least 3 months. Results Between September 1997 and January 2020, 21 patients (focal segmental glomerulosclerosis: 12, minimal change nephrotic syndrome: 9, men: 67%, median age: 34 years) were identified. At last follow-up (12 months), 7 of 21 patients were in complete or partial remission. Remission was associated with older age (51 vs. 30 years, P = 0.05), lower proteinuria (3.9 vs. 7.3 g/d, P = 0.03), and lower estimated glomerular filtration rate (eGFR) (28.0 vs. 48.5 ml/min per 1.73 m 2 , P = 0.05) at apheresis. The need for dialysis before apheresis (odds ratio [OR] 22.0 [1.00–524], P = 0.026), age ≥50 years (OR: 22.6 [1.00–524], P = 0.006), a marked (>4.5 g/d) decrease in proteinuria (OR: 9.17 [1.15–73.2], P = 0.041), and a short (<12 months) time between diagnosis and apheresis (OR: 10.8 [1–117], P = 0.043) were significantly associated with remission. Three of 7 patients in remission who were initially on dialysis became dialysis-free; by contrast, none of the 14 patients without remission was initially on dialysis, but 5 of 14 had become dialysis-dependent ( P = 0.01). Conclusion Apheresis may result in remission in adult patients with refractory INS, particularly in those at risk of renal failure, with limited sensitivity to medical treatments, if apheresis is initiated within a year of diagnosis.

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Section: Discussionmentioning

confidence: 99%

“…Most of the available data originate from Japan and relate to patients with low-density lipoprotein (LDL)-apheresis. 12 , 13 , 14 , 15 , 16 …”

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confidence: 99%

Apheresis in Adult With Refractory Idiopathic Nephrotic Syndrome on Native Kidneys

Moret

Ganea

Dao

et al. 2021

Kidney International Reports

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“…All those RNAs, including EBER1 and EBER2 are powerful TLR3 agonists [ 35 ] susceptible to interact with TLR3 expressed on the podocyte and to affect podocyte integrity in a similar way as previously described by Shimada and Garin. Moreover, the transmission of proteinuria to mice by antiUCHL1 IgG antibodies purified from a relapsing patient [ 36 ], as well as the efficacy of immunoglobulin adsorption in the multiresistant forms of INS [ 37 ] are also relevant arguments to involve antibodies as the second podocyte hit that leads to massive proteinuria. It turns out that EBNA1 and UCHL1 share two short peptide sequences located in the immunogen domain of EBNA1 and in close vicinity at the surface of UCHL1 (blast in https://web.expasy.org/sim/ ; reference of protein sequences: YP_401677.1 and NP_004172.2 respectively).…”

Section: Discussionmentioning

confidence: 99%

A global antiB cell strategy combining obinutuzumab and daratumumab in severe pediatric nephrotic syndrome

et al. 2020

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Background Steroid-sensitive nephrotic syndrome (SSNS) is, in most patients, a chronic disease with 80% experiencing at least one relapse after first flare. B cell depletion using rituximab is effective in preventing relapse in steroid-dependent (SDNS) patients but fails to maintain long-term remission following B cell recovery, possibly due to development of autoreactive long-lived plasma cells. We investigated sequential combination of antiCD20 antibody targeting all B cell subsets, and antiCD38 antibody with high plasma cell cytotoxicity in patients with uncontrolled SDNS after failure of one or several attempts at B cell depletion. Methods Fourteen patients with median disease duration 7.8 years received 1000 mg/1.73 m 2 obinutuzumab followed by 1000 mg/1.73 m 2 daratumumab 2 weeks later. Oral immunosuppression was discontinued within 6 weeks, and biological monitoring performed monthly until B cell recovery. Results Median age at treatment was 11.0 [IQR 10.4–14.4] years. B cell depletion was achieved in all patients, and B cell reconstitution occurred in all at median 9.5 months after obinutuzumab injection. After median follow-up 20.3 months (IQR 11.5–22.6), 5/14 patients relapsed including 4 within 100 days following B cell repletion. Relapse-free survival was 60% at 24 months from obinutuzumab infusion. Mild infusion reactions were reported in 3/14 patients during obinutuzumab and 4/14 during daratumumab infusions. Mild transient neutropenia (500–1000/mm 3 ) occurred in 2/14 patients. Intravenous immunoglobulins were given to 12/14 patients due to hypogammaglobulinemia. Low IgA and IgM levels were noted in 8 and 14 patients, respectively. No severe infection was reported. Conclusion Global antiB cell strategy combining obinutuzumab and daratumumab induces prolonged peripheral B cell depletion and remission in children with difficult-to-treat SDNS. Electronic supplementary material The online version of this article (10.1007/s00467-020-04811-0) contains supplementary material, which is available to authorized users.

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“…Raina et al reported on 11 steroid/cyclosporine-resistant children with INS where apheresis induced remission in 64%, i.e., proteinuria decreased from 9.7+/−2.5 to 1 +/0.5 g/d [11]. Similarly, Nattes et al [12] gave IA (10 sessions over a 2-week period) to 14 steroid/calcineurin-inhibitor-resistant children with INS (58% were FSGS); this resulted in full or partial remission in 50% and 15% of cases, respectively.…”

Section: Discussionmentioning

confidence: 99%

Apheresis Therapy for Steroid-Resistant Idiopathic Nephrotic Syndrome: Report on a Case Series

Bennani

Jouve

Noble

et al. 2019

Case Reports in Nephrology

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Idiopathic nephrotic syndrome (INS) represents 15%–30% of adulthood glomerulopathies. Corticosteroids have been the main treatment for decades and are effective in 70% of minimal-change disease patients and ~30% of focal segmental glomerulosclerosis patients. Multidrug-resistant (steroids, calcineurin-inhibitors, cyclophosphamide, mycophenolate-mofetil, rituximab) idiopathic nephrotic syndrome is a major therapeutic challenge in nephrology. Apheresis (double-filtration plasmapheresis or semi specific immunoadsorption) could act by eliminating the circulating factor (apolipoproteinA1b, solubleCD40L, suPAR) increasing glomerular permeability seen in INS. The aim of the study was to report the outcome of three patients with multidrug-resistant INS treated successfully with apheresis.

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Remission of proteinuria in multidrug‐resistant idiopathic nephrotic syndrome following immunoglobulin immunoadsorption

Abstract: Patients with multidrug-resistant idiopathic nephrotic syndrome can be successfully led into remission by IgIA prior to reaching end-stage renal disease. However, IgIA does not suppress the need for heavy additional immunosuppression to control INS in most cases.

Cited by 3 publications

References 19 publications

Apheresis in Adult With Refractory Idiopathic Nephrotic Syndrome on Native Kidneys

Apheresis in Adult With Refractory Idiopathic Nephrotic Syndrome on Native Kidneys

A global antiB cell strategy combining obinutuzumab and daratumumab in severe pediatric nephrotic syndrome

Apheresis Therapy for Steroid-Resistant Idiopathic Nephrotic Syndrome: Report on a Case Series

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