Profile of Indian Patients With Membranous Nephropathy

Subramanian, Priyadarsani; Kumar, Hitesh; Tiwari, Bipin; Barwad, Adarsh; Bagchi, Soumita; Bagga, Arvind; Agarwal, Sanjay Kumar; Dinda, Amit Kumar; Singh, Geetika

doi:10.1016/j.ekir.2020.06.024

Cited by 13 publications

(10 citation statements)

References 28 publications

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“…Only 2 cases were positive for THSD7A, neither one was associated with neoplasms, though 1 was identified in a case of MLN. Similar isolated cases of THSD7A-positive MLN have been reported in the literature [40,43]. NELL1, a newly identified and second most common target antigen in pMN, has also been linked with malignancies in onethird of cases and rarely HSCT and lipoic acid supplementation [44][45][46].…”

Section: Discussionsupporting

confidence: 68%

“…Conversely, 2 cases of MLN were positive for a marker of pMN: 1 with PLA2R and 1 with THSD7A. Although antibodies to target antigens associated with pMN are only rarely encountered in sec-Nephron 2023;147:424-433 DOI: 10.1159/000529437 ondary forms of MN, including MLN, multiple studies have reported the presence of PLA2R (both by IHC and serology) in up to 18.9% of MLN patients and in a subset associated with other ADs such as psoriasis, HCV, HBV, sarcoidosis, and neoplasms [38][39][40][41][42]. In our PLA2R-positive pMN cohort, 2 patients had neoplasms (both prostate CA) and 2 had chronic GVHD.…”

Section: Discussionmentioning

confidence: 99%

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The Clinicopathologic Spectrum of Membranous Nephropathy with Lupus-Like Features

Choung

Moore

et al. 2023

Nephron

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Introduction: The pathologic features of membranous lupus nephritis (MLN) are occasionally encountered in secondary membranous nephropathy (sMN) without overt clinical evidence of SLE. Moreover, some sMN with lupus-like features (LL-MN) have a clinical presentation more typical of primary membranous nephropathy (pMN). Based on the confounding clinical and pathologic presentation, it is unclear how to categorize and treat these patients. Methods: We performed immunohistochemical staining for recently discovered target antigens associated with MN -NELL-1, THSD7A, and EXT1/2, and compared the clinicopathologic presentation of patients with LL-MN to those with pMN and MLN. Results: From 2015-2020, there were 21 patients with MLN and 99 with MN, of which 59% were diagnosed pMN and 41% sMN. 44% of sMN showed lupus-like features (LL-fx). All LL-MN were negative for PLA2R and NELL1, but 12% were positive for EXT1/2. 50% of LL-MN had an identifiable systemic disease, of which 56% were autoimmune disease (AD) and 44% infection. Compared to pMN, LL-MN had higher incidence of underlying AD (p=0.02). Within pMN, 24% also had LL-fx (LL-pMN), and all but 1 were PLA2R-(78%) or NELL1-positive (15%). Only 5% of pMN had an AD, 66% of which showed LL-fx. Most idiopathic LL-MN were treated and behaved clinically similarly to pMN. There were no differences in outcome in terms of progression towards ESRD or mortality between LL-MN versus pMN and MLN. Conclusion: LL-MN appears to have a significant association with underlying AD and has a subset showing EXT1/2 positivity, whereas most LL-pMN and idiopathic LL-MN likely represent an atypical pathologic presentation of pMN.

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Section: Discussionsupporting

confidence: 68%

Section: Discussionmentioning

confidence: 99%

The Clinicopathologic Spectrum of Membranous Nephropathy with Lupus-Like Features

Choung

Moore

et al. 2023

Nephron

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“…Although it is widely accepted that children presenting with nephrotic syndrome likely have minimal change disease and that empirical treatment with corticosteroids without a kidney biopsy is the standard of care, a recent publication from India reported 32 pediatric patients with nephrotic syndrome who received a trial of steroids prior to biopsy and were eventually diagnosed with membranous nephropathy. Four of the 25 patients with primary membranous nephropathy in this cohort had positive PLA2R serology (25), highlighting the usefulness of this biomarker even in the pediatric population.…”

Section: Discussionmentioning

confidence: 84%

Noninvasive Diagnosis of PLA2R-Associated Membranous Nephropathy

Bobart

Han

Tehranian

et al. 2021

CJASN

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Background and objectivesKidney biopsy is the current gold standard to diagnose membranous nephropathy. Approximately 70%–80% of patients with primary membranous nephropathy have circulating anti-phospholipase A2 receptor antibodies. We previously demonstrated that in proteinuric patients with preserved eGFR and absence of associated conditions (e.g., autoimmunity, malignancy, infection, drugs, and paraproteinemia), a positive anti-phospholipase A2 receptor antibody test by ELISA and immunofluorescence assay confirms the diagnosis of membranous nephropathy noninvasively. These data have not been externally validated.Design, setting, participants, & measurementsThe clinical and pathologic characteristics of patients with a positive anti-phospholipase A2 receptor antibody test at the Mayo Clinic, the University Hospital Vall D’Hebron (Barcelona), and the Columbia University Medical Center (New York) were retrospectively reviewed. Biopsy findings and presence or absence of a potential associated condition were assessed.ResultsFrom a total of 276 patients with positive anti-phospholipase A2 receptor serology, previously reported patients (n=33), kidney transplant recipients (n=9), pediatric patients (n=2), and patients without kidney biopsy (n=69) were excluded. Among the 163 remaining patients, associated conditions were identified in 47 patients, and 15 patients had diabetes mellitus. All 101 patients of the final cohort had a primary diagnosis of membranous nephropathy on kidney biopsy. In the 79 patients with eGFR≥60 ml/min per 1.73 m2, none of the biopsy findings altered diagnosis or management. Among the 22 patients with decreased eGFR, additional findings included superimposed acute interstitial nephritis (n=1).ConclusionsIn patients with preserved eGFR and absence of associated conditions or diabetes, a positive anti-phospholipase A2 receptor test by either ELISA >20 RU/ml or a positive immunofluorescence assay confirms the diagnosis of membranous nephropathy, precluding the requirement for a kidney biopsy.

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“…25 of the 32 pediatric patients aged between 6 to 17 years were pMN, and 7 were sMN. 11 of the 25 pMN (44%) were PLA2R-positive on tissue staining while serum was available for testing in 5 of them, 4 of which were positive (also positive on tissue staining) ( 33 ). None of the secondary MN were positive.…”

Section: Pla2r-associated Mnmentioning

confidence: 99%

“…Furthermore, the level of THSD7A antibodies is associated with clinical remission, often decreasing with immunotherapy. In the retrospective study conducted in India with 184 adults and 32 pediatric patients, THSD7A was tested for in 29 pediatric cases and was positive in only one (a 17 years old pMN patient) ( 33 ).…”

Section: Thsd7a-associated Mnmentioning

confidence: 99%

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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Profile of Indian Patients With Membranous Nephropathy

Cited by 13 publications

References 28 publications

The Clinicopathologic Spectrum of Membranous Nephropathy with Lupus-Like Features

The Clinicopathologic Spectrum of Membranous Nephropathy with Lupus-Like Features

Noninvasive Diagnosis of PLA2R-Associated Membranous Nephropathy

Pediatric membranous nephropathy: In the novel antigens era

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