Primary membranous nephropathy in children and adolescents: a single-centre report from South Asia

Ramachandran, Raja; Nayak, Saurabh; Kumar, Vinod; Kumar, Ashwani; Agrawal, Neha; Bansal, Ritika; Tiewsoh, Karalanglin; Nada, Ritambhra; Rathi, Manish; Kohli, Harbir Singh

doi:10.1007/s00467-020-04798-8

Cited by 9 publications

(8 citation statements)

References 33 publications

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“…Literature about immunotherapy management in pediatric patients with MN is rare. Lee et al ( 12 ) reported a clinical remission rate of 68% in 19 pediatric patients with MN receiving immunotherapy management; Ramachandran et al ( 64 ) demonstrated in an uncontrolled study of 48 pediatric patients with MN that 2/3 cases responded to immunosuppressive therapy. Unlike adult MN patients, renal biopsy is not usually performed in pediatric population with steroid-sensitive NS.…”

Section: Treatment Of Mnmentioning

confidence: 99%

“…It showed two-thirds patients responded to immunotherapy. And among all of the patients 60% patients treated with rituximab, it is useful as a first-line and rescue therapy ( 64 ). Two cases of adolescent MN with elevated anti-PLA2R levels and nephrotic-range proteinuria who did not achieve remission with steroids were reported successfully treated with rituximab ( 68 ).…”

Section: Treatment Of Mnmentioning

confidence: 99%

“…Nevertheless, the optimal dosing and timing of rituximab in pediatric patients have not been established. Some patients received 1 g rituximab (days 0 and 15), some received 375 mg/m2 (×4 weekly doses), and others underwent CD19-targeted rituximab therapy ( 64 ). Although rituximab is effective, the side effects in pediatric patients should be considered, including severe infections, neutropenia and hypogammaglobulinemia.…”

Section: Treatment Of Mnmentioning

confidence: 99%

“…47 (97.91%) cases received immunosuppressive therapy, and over two-thirds of the patients respond to immunosuppressive therapy (However, it is unknown whether immunosuppressive agents were used in the 4 cases with non-nephrotic proteinuria). And the authors suggested that the choice of immunosuppressive therapy should be based on the patients’ characteristics and nephrologists’ experience ( 64 ). In a retrospective study including 37 children with MN (all of them have received a full dose of steroids for up to six weeks), 19 of whom were given other immunosuppressive agents after the renal biopsy, 7 continued steroids-alone therapy, and only 11 received no immunosuppressive therapy.…”

Section: Treatment Of Mnmentioning

confidence: 99%

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Pediatric membranous nephropathy: In the novel antigens era

Huang

Liu

et al. 2022

Front. Immunol.

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Membranous nephropathy (MN) falls within the scope of a glomerular disease. MN exhibits subepithelial immune- complex deposition and capillary wall thickening which could occur in all age groups. In comparison with adult patients with MN, MN in pediatric population has a lower incidence and more secondary factors (e.g., systemic lupus erythematosus, infection, malignancy, or drug toxicity). Two target antigens for the immune complexes, PLA2R (identified in 2009) and THSD7A (in 2014), found in previous studies and first presented in adult MN, are found in pediatric patients suffering from MN and their antibodies are now an effective tool for diagnosis and monitoring in children and adolescents. Several novel antigens have been identified (e.g., EXT1/EXT2, NELL1, Sema3B, PCDH7, HTRA1, and NCAM1) over the past few years. Each of them represents different clinical and pathologic findings. In-depth research should be conducted to gain insights into the outcomes and pathophysiology of the above novel antigen-associated MN. Targeted treatment opinions for different novel antigen-related MN are under development both in adults and pediatric patients.

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Section: Treatment Of Mnmentioning

confidence: 99%

Section: Treatment Of Mnmentioning

confidence: 99%

Section: Treatment Of Mnmentioning

confidence: 99%

Section: Treatment Of Mnmentioning

confidence: 99%

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Pediatric membranous nephropathy: In the novel antigens era

Huang

Liu

et al. 2022

Front. Immunol.

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“…Membranous nephropathy (MN) is an autoimmune kidney disease that is a common cause of nephrotic syndrome in adults, and its incidence has increased gradually in children over the past decades [ 1 – 4 ]. It is characterized by massive proteinuria (>3.5 g/day) and diffuse thickening of the glomerular basement membrane, due to the presence of immune deposits [ 1 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

Clinical Evaluation of Antiphospholipase A2 Receptor IgG4 level and Its IgG4-to-IgG Ratio Based on Quantitative Immunoassays in Idiopathic Membranous Nephropathy

Zhang

Huang

et al. 2022

BioMed Research International

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Background. Phospholipase A2 receptor (PLA2R), located at the membrane of glomerular podocyte, is the major autoantigen of idiopathic membranous nephropathy (IMN), and its antibodies with a predominant IgG4 subclass lead to pathological lesions. Further studies could be performed to validate the clinical values of PLA2R-IgG, PLA2R-IgG4, and PLA2R-IgG4-to-IgG ratios, as ultrasensitive and quantitative immunoassays for PLA2R antibodies have been well established in our previous work. Methods. A cohort of 58 IMN patients, 30 of whom were followed from 3 to 42 months, was assessed for serum PLA2R-IgG and -IgG4 levels, and the ratio of PLA2R-IgG4/-IgG combined with relative clinicopathological indicators. Results. Serum PLA2R-IgG4 level was significantly correlated with glomerular PLA2R staining. In addition, it was strongly correlated with PLA2R-IgG and its ratio. PLA2R-IgG and -IgG4 levels were both correlated with high-density lipoprotein and erythrocyte sedimentation rates. The ratio at the first diagnosis can predict the remission, and its efficacy overmatched PLA2R-IgG4. In the survival curves, negative results for the ratio or PLA2R-IgG4 at the first diagnosis demonstrated higher remission rates. Conclusion. Serum PLA2R-IgG4 concentration may replace renal PLA2R immunohistochemistry in IMN diagnosis. We propose that the PLA2R-IgG4-to-IgG ratio and PLA2R-IgG4 could be novel indicators for remission prediction in clinical practice.

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Membranous Nephropathy

Khalid

Beck²

2023

Pediatric Kidney Disease

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Primary membranous nephropathy in children and adolescents: a single-centre report from South Asia

Cited by 9 publications

References 33 publications

Pediatric membranous nephropathy: In the novel antigens era

Pediatric membranous nephropathy: In the novel antigens era

Clinical Evaluation of Antiphospholipase A2 Receptor IgG4 level and Its IgG4-to-IgG Ratio Based on Quantitative Immunoassays in Idiopathic Membranous Nephropathy

Membranous Nephropathy

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