scholarly journals Cyclical cyclophosphamide and steroids is effective in resistant or relapsing nephrotic syndrome due to M-type phospholipase A2 receptor–related membranous nephropathy after tacrolimus therapy

2016 ◽  
Vol 89 (6) ◽  
pp. 1401-1402 ◽  
Author(s):  
Raja Ramachandran ◽  
Vinod Kumar ◽  
Vivekanand Jha
Keyword(s):  
Nephrotic Syndrome ◽  
Phospholipase A2 ◽  
Membranous Nephropathy ◽  
Phospholipase A2 Receptor ◽  
Tacrolimus Therapy

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 Circulating anti-phospholipase A2 receptor antibodies as a diagnostic and prognostic marker in Greek patients with idiopathic membranous nephropathy – a retrospective cohort study

2019 ◽  
Vol 57 (2) ◽  
pp. 141-150
Author(s):  
Simella Provatopoulou ◽  
Dimitra Kalavrizioti ◽  
Maria Stangou ◽  
Maria-Nikoleta Kouri ◽  
Pantellitsa Kalliakmani ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Complete Remission ◽  
Phospholipase A2 ◽  
Disease Activity ◽  
Membranous Nephropathy ◽  
Clinical Status ◽  
Immunosuppressive Treatment ◽  
Idiopathic Membranous Nephropathy ◽  
Phospholipase A2 Receptor

Abstract Introduction. Circulating autoantibodies against phospholipase A2 receptor (anti-PLA2R) are recognized as key elements in the pathogenesis of idiopathic membranous nephropathy. In current clinical practice, they are increasingly gaining attention as novel tools for diagnosis and disease monitoring. We investigated the diagnostic and prognostic utility of anti-PLA2R antibody measurements in Greek patients with biopsy-proven membranous nephropathy. Methods. Anti-PLA2R levels were measured in serum samples from 33 patients at diagnosis using ELISA and were associated with treatment outcome. Moreover, serial anti-PLA2R measurements were performed in 15 patients under different clinical conditions and level alterations were correlated with disease activity. Results. Positive anti-PLA2R antibodies at diagnosis were found in 16 of 33 patients (48.5%). Anti-PLA2R levels were independently associated with the achievement of complete remission of nephrotic syndrome after immunosuppressive treatment compared to partial remission (p = 0.02, R2 = 0.265, 95%CI -0.019 to -0.0003). Higher detectable antibody levels at diagnosis were correlated with higher proteinuria levels (r = 0.813, p = 0.0001, 95%CI 0.532 to 0.933) and lower eGFR at the end of follow-up (r = -0.634, p = 0.0083, 95%CI -0.86 to -0.202). Serial antibody measurements during follow-up showed that anti-PLA2R titers were significantly reduced at the end of treatment after complete remission was achieved, remained low under sustained clinical remission, and increased during relapse. Conclusions. Our findings confirm the usefulness of anti-PLA2R measurements in the diagnosis of idiopathic membranous nephropathy. Low levels of anti-PLA2R antibodies at diagnosis are predictive of complete remission of nephrotic syndrome following immunosuppressive treatment. Serial anti-PLA2R measurements correlate well with clinical status throughout the follow-up period and could be used routinely for monitoring of disease activity and treatment planning.

scholarly journals A case report of breast cancer and membranous nephropathy with positive anti phospholipase A2 receptor antibodies

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
David Mathew ◽  
Sanjana Gupta ◽  
Neil Ashman
Keyword(s):  
Breast Cancer ◽  
Nephrotic Syndrome ◽  
Phospholipase A2 ◽  
Membranous Nephropathy ◽  
Ductal Carcinoma ◽  
Case Series ◽  
Solid Organ ◽  
First Case ◽  
Phospholipase A2 Receptor ◽  
Solid Organ Malignancy

Abstract Background Testing for antibodies against podocyte phospholipase A2 receptor-1 (PLA2R) allows clinicians to accurately identify primary membranous nephropathy (MN). Secondary MN is associated with a spectrum of pathology including solid organ malignancy. PLA2R positivity in these patients occurs, although no case of PLA2R-positive MN has been definitively linked to cancer. Case presentation We describe a case of biopsy-proven PLA2R-positive MN, in whom invasive ductal carcinoma of the breast was discovered. The patient underwent surgery and adjuvant chemotherapy (including cyclophosphamide) and went into a sustained complete remission of her nephrotic syndrome. Discussion and conclusions Case series have reported PLA2R positivity in patients with solid organ malignancy associated MN. Our case is unusual as it is a breast malignancy, and the patients nephrotic syndrome and anti-PLA2Rab titres improved with treatment of the cancer. Here we report, to the best of our knowledge, the first case of oestrogen receptor-2 positive breast cancer associated with PLA2R positive MN in a young lady that was treated successfully by treating the malignancy.

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

2018 ◽  
Author(s):  
Evgeny Farber
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 Predictive values of serum phospholipase A2 receptor antibody and urinary IgG, α1- macroglobulin in patients with idiopathic membranous nephropathy and nephrotic syndrome

2020 ◽  
Author(s):  
Lili Liu ◽  
Haitao Wang ◽  
Ban Zhao ◽  
Xin Liu ◽  
Ying Sun ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Phospholipase A2 ◽  
Membranous Nephropathy ◽  
Multivariate Regression ◽  
Idiopathic Membranous Nephropathy ◽  
Renal Outcome ◽  
Predictive Values ◽  
Receptor Antibody ◽  
Phospholipase A2 Receptor

Abstract BackgroundThe biomarkers predicting long-term outcome of idiopathic membranous nephropathy (IMN) with nephrotic syndrome (NS) remains indeterminacy. We conducted this study to evaluate the different features between phospholipase A2 receptor (PLA2R)-associated and non-PLA2R-associated IMN, and to explore the association between serum PLA2R antibody (PLA2R-Ab), urinary immunoglobulin G (UIgG), urinary α1-macroglobulin (Uα1m) and renal outcomes in patients with idiopathic membranous nephropathy (IMN) and nephrotic syndrome (NS). MethodsIMN patients who were biopsy-proven and presenting NS were retrospectively recruited for the present study. Serum PLA2R-Ab levels were detected by enzyme-linked immunosorbent assay (ELISA) kits, and values over 20 RU/mL was considered positive. UIgG) and Uα1m were measured by immunonephelometry and corrected by urinary creatinine. The clinicopathologic features, remission and renal outcome were compared between the PLA2R-associated and non-PLA2R-associated IMN patients. Furthermore, the predictive values of biomarkers (PLA2R-Ab, UIgG/Cr and Uα1m/Cr) for remission and renal outcome were assessed by multivariate regression. The renal endpoint was defined as progression to end stage kidney disease (ESRD) or estimated glomerular filtration rate (eGFR) decline ≥50% of baseline. ResultsA total of 111 IMN patients were enrolled this study, and 81 (73.0%) of them were PLA2R-associated. The mean age, 24-hour proteinuria and eGFR showed no difference between PLA2R-associated and non-PLA2R-associated groups (p>0.05). However, PLA2R-associated IMN patients had significantly higher UIgG/Cr (17.78 vs. 9.82 mg/g; median, p=0.001) and Uα1m (0.339 vs 0.202 mg/g; median, p<0.001) when compared to non-PLA2R-associated patients. Histologically, the PLA2R-associated group represented more proportion of patients with acute tubular necrosis (ATN) (27.16% vs. 3.33%, P=0.006) and glomerular C3 deposits (88.89% vs. 70.00%, P=0.016) than the non-PLA2R-associated group. During a median follow-up of 40 months (range 9 to 92), non-PLA2R-associated patients had significantly higher remission rate at the 6th and 12th month and end of follow-up, even after adjusting for the use of immunosuppressor. Furthermore, 11 (13.6%) patients reaching renal endpoint were all PLA2R-associated IMN. Multivariate regression analysis represented that baseline serum PLA2R-Ab titer was an independent predictor of remission (OR, 1.002; 95% confidence interval [CI] 1.001 to 1.004; p=0.002) and renal outcome (HR, 1.002; 95% CI 1.001-1.003, p= 0.004). Receiver operating characteristic (ROC) showed that serum PLA2R-Ab titer >216.93 RU/ml (AUC=0.778, p=0.003), UIgG/Cr >15.76mg/g (AUC=0.758, p=0.005) and Uα1m/Cr >0.3042mg/g (AUC=0.738, p=0.010) predicted renal failure in patients with IMN and NS. Kaplan-Meier curves indicated that subjects with combination of all three high biomarkers had significantly shorter renal survival (log rank p=0.007) than subjects with ≤2 high biomarkers. ConclusionHigh PLA2R-Ab levels is poor prognosis predictor of IMN in addition to proteinuria. In addition, combination of multiple factors (PLA2R-Ab, UIgG and Uα1m) represents a stronger predictive power. These findings suggested the potential different pathogenesis and progression in IMN with NS. Keywords: idiopathic membranous nephropathy, phospholipase A2 receptor antibody, urinary IgG, urinary α1- macroglobulin, nephrotic syndrome.

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