scholarly journals Phospholipase A2 Receptor-Positive Idiopathic Membranous Glomerulonephritis with Onset at 95 Years: Case Report

2016 ◽  
Vol 6 (2) ◽  
pp. 76-82 ◽  
Author(s):  
Keiichi Kubota ◽  
Junichi Hoshino ◽  
Toshiharu Ueno ◽  
Koki Mise ◽  
Ryo Hazue ◽  
...  
Keyword(s):  
Phospholipase A2 ◽  
Membranous Glomerulonephritis ◽  
Underlying Disease ◽  
Immunoglobulin G4 ◽  
Protein Excretion ◽  
Phospholipase A2 Receptor ◽  
Limb Edema ◽  
Bilateral Lower Limb ◽  
Idiopathic Membranous Glomerulonephritis ◽  
Stage 1

A 95-year-old woman was admitted to our hospital for evaluation of bilateral lower-limb edema persisting for 3 months. Serum creatinine was 1.55 mg/dl, and urinary protein excretion was 9.1 g/day. Renal biopsy revealed stage 1 membranous glomerulonephritis (MGN) with immunoglobulin G4-dominant staining. This patient did not have any underlying disease such as infection with hepatitis B or C virus or malignancy, and anti-phospholipase A2 receptor (PLA2R) antibody was detected in the serum. Accordingly, idiopathic MGN was diagnosed. Corticosteroid therapy was avoided, but hemodialysis was required to treat generalized edema. The patient is currently doing well. This is the oldest reported case of idiopathic MGN with positivity for anti-PLA2R antibody.

scholarly journals Enigma (partially) resolved: phospholipase A2 receptor is the cause of “idiopathic” membranous glomerulonephritis

2015 ◽  
Vol 309 (12) ◽  
pp. F1000-F1002 ◽  
Author(s):  
Luan D. Truong ◽  
Surya V. Seshan
Keyword(s):  
Plasma Membrane ◽  
Transmembrane Protein ◽  
Membranous Glomerulonephritis ◽  
Prediction Of Outcome ◽  
Protein Excretion ◽  
Immune Mediated ◽  
Phospholipase A2 Receptor ◽  
Idiopathic Membranous Glomerulonephritis ◽  
Diagnosis Therapy ◽  
Antigen Antibody

Membranous glomerulonephritis (MGN) is a very significant kidney disease. It is one of the frequent causes of heavy protein excretion in urine. MGN is thought to be an immune-mediated disease caused by glomerular deposition of antigen-antibody complexes. The pathogenic antigen, however, has been an enigma until recently. It was discovered in 2009 that phospholipase A2 receptor (PLA2R), a normal transmembrane protein in podocyte plasma membrane, is the antigen causing MGN. Within 5 yr of its discovery, this seminal finding has leaded to novel insights into the treatment of this disease including diagnosis, therapy, and prediction of outcome. This finding also paves the way for fundamental studies on how and why autoimmunity against PLA2R develops. The discovery of PLA2A as the cause of “idiopathic” MGN after a half century of speculation, followed by further fundamental insights with such an expedient and successful application in patient care, embodies the elegance of science at its junction with society. This perspective traces the story of this remarkable discovery.

Validation of a phospholipase A2 receptor antibody ELISA in an Australian cohort with membranous glomerulonephritis

Pathology ◽  
2016 ◽  
Vol 48 (3) ◽  
pp. 242-246 ◽  
Author(s):  
Lawrence Ong ◽  
Roger Silvestrini ◽  
Jeremy Chapman ◽  
David A. Fulcher ◽  
Ming Wei Lin
Keyword(s):  
Phospholipase A2 ◽  
Membranous Glomerulonephritis ◽  
Receptor Antibody ◽  
Phospholipase A2 Receptor ◽  
Australian Cohort ◽  
Antibody Elisa

scholarly journals Phospholipase A2 receptor staining is absent in many kidney biopsies with early-stage membranous glomerulonephritis

2016 ◽  
Vol 89 (6) ◽  
pp. 1402-1403 ◽  
Author(s):  
Margaret S. Ryan ◽  
Anjali A. Satoskar ◽  
Gyongyi M. Nadasdy ◽  
Sergey V. Brodsky ◽  
Jessica A. Hemminger ◽  
...  
Keyword(s):  
Phospholipase A2 ◽  
Early Stage ◽  
Membranous Glomerulonephritis ◽  
Phospholipase A2 Receptor

scholarly journals Successful foetal outcome in a patient with primary membranous nephropathy and circulating anti-phospholipid antibodies: A case report

2018 ◽  
Keyword(s):  
Nephrotic Syndrome ◽  
Phospholipase A2 ◽  
Membranous Nephropathy ◽  
Membranous Glomerulonephritis ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Phospholipase A2 Receptor ◽  
Foetal Outcome ◽  
Circulating Autoantibodies ◽  
Patient’S History

There is little information about pregnancy outcomes in patients with active membranous nephropathy (MN), especially those with circulating autoantibodies to M-type phospholipase A2 receptor (PLA2R), the major autoantigen in primary MN. Membranous glomerulonephritis (MGN) represents an immunologically mediated disease characterized by deposition of immune complexes in the glomerular subepithelial space, frequently associated with circulating M-type phospholipase A2 receptor. Nephrotic syndrome (massive proteinuria and hypoalbuminemia) at diagnosis predicts poor prognosis. Pregnancy with active MGN is high risk for foetal loss, intrauterine growth restriction, and pre-eclampsia, and may worsen maternal renal function, especially with the presence of antiphospholipid antibody syndrome (APLA). We report a 23-year-old gravida in her first pregnancy, suffering from MGN and severe nephrotic syndrome, complicated by APLA syndrome. The patient was treated with enoxaparin, aspirin azathioprine, and Prednisone for a short time, in addition to furosemide and albumin intravenously. She was delivered at 30 weeks due to deteriorating maternal and foetal conditions. A successful neonatal and maternal outcome was achieved in this case. The patient's history revealed thrombocytopenia and APLA syndrome and continues to be treated chronically with enoxaparin. Kidney biopsy performed after delivery showed membranous MGN stage II-III. Herein, we present a case of successful pregnancy and foetal outcome in a young woman with APLA syndrome and MN. Keywords: Membranous GN, Nephrotic Syndrome, Anti-Phospholipid Antibodies.

scholarly journals Analysis of Glomerular IgG Subclasses Switch in Idiopathic Membranous Nephropathy Classified by Glomerular Phospholipase A2 Receptor Antigen and Serum Antibody

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Hao-yuan Cui ◽  
Chao Li ◽  
Hang Li ◽  
Yu-bing Wen ◽  
Lin Duan ◽  
...  
Keyword(s):  
Phospholipase A2 ◽  
Membranous Nephropathy ◽  
Serum Antibody ◽  
Chinese Patients ◽  
Idiopathic Membranous Nephropathy ◽  
Igg Subclass ◽  
Electron Microscopic ◽  
Phospholipase A2 Receptor ◽  
Stage 1

Background. The role of IgG subclass in idiopathic membranous nephropathy (IMN) was unclarified. Recent study found IgG subtype switches from IgG1 to IgG4 in the early pathological stage in IMN. The profile of IgG subclass in phospholipase A2 receptor- (PLA2R-) related and PLA2R-unrelated IMN was unrevealed. Our study is aimed at testifying whether IgG subclass switch existed in PLA2R-related and PLA2R-unrelated IMN, respectively. Methods. Our study retrospectively enrolled 157 Chinese patients with biopsy-confirmed IMN between September 2017 and November 2019. We measured glomerular PLA2R antigen and serum anti-PLA2R antibody to classify the patients into PLA2R-related ( n = 132 ) and PLA2R-unrelated ( n = 25 ) subgroup. We evaluated glomerular IgG subclass by immunofluorescence (IF) predominance. Our study defined IgG subclass deposition as predominant if the IF score was higher than the other three and ≥1 +, or as codominant if the IF intensity was equal to any other and ≥1 +. We explored the relationship between IF predominance of glomerular IgG subtype and electron microscopic (EM) stages of IMN. Results. We did not find statistical difference of predominant or codominant rate (pre/co-rate) among EM stages in any subclass ( P > 0.05 ). Pre/co-rate of IgG3 linearly associated with EM stage in total and PLA2R-related subgroup ( P = 0.044 , P = 0.013 ). PLA2R-related subgroup showed higher IgG4 intensity ( 2.1 ± 0.6 vs. 1.6 ± 0.7 , P = 0.001 ) and pre/co-rate of IgG4 in stage 1 (97% vs. 57%, P = 0.015 ) than PLA2R-unrelated group. We found no difference of IgG subclass pre/co-rate in different EM stages or linear association between pre/co-rate of IgG1, IgG2, IgG4, and EM stages ( P > 0.05 ). Conclusions. Pre/co-rate of IgG3 declined with EM stage in total and PLA2R-related subgroup. We did not find IgG subclass switches from IgG1 to IgG4 in either IMN patients or subgroups.

scholarly journals Secondary membranous nephropathy in a patient with myasthenia gravis without thymic disease, and partial remission induced by adrenocorticotropic hormone therapy

2019 ◽  
Vol 7 ◽  
pp. 2050313X1986976 ◽  
Author(s):  
Ramy M Hanna ◽  
Farid Arman ◽  
Umut Selamet ◽  
William D Wallace ◽  
Marina Barsoum ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Phospholipase A2 ◽  
Adrenocorticotropic Hormone ◽  
Case Reports ◽  
Membranous Glomerulonephritis ◽  
Autoimmune Disorders ◽  
African American Female ◽  
Phospholipase A2 Receptor ◽  
Secondary Membranous Nephropathy ◽  
Receptor Antibodies

Membranous glomerulonephritis is the most common glomerular disease in adults. Its primary form has been characterized with formation of phospholipase A2 receptor antibodies. Malignancy, infections, and autoimmune disorders are the most common causes of secondary membranous glomerulonephritis. We present a case of a 55-year-old African American female who presented with nephrotic range proteinuria and diagnosed with secondary membranous glomerulonephritis based on distinct pathological features on kidney biopsy and absence of serum phospholipase A2 receptor antibodies. She initially underwent extensive workup for malignancies, infections, and common autoimmune disorders which were all negative. Her proteinuria remained resistant to steroid treatment and she was treated with subcutaneous adrenocorticotropic hormone injections. Meanwhile, she was also diagnosed with the anti-muscle specific kinase antibody variant of myasthenia gravis. In literature, there are few case reports of myasthenia gravis as a cause of secondary membranous glomerulonephritis. In our case, the lack of other inciting factors also suggested this association.

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