Validation of phospholipase A2 receptor direct immunofluorescence staining in the diagnosis of primary membranous glomerulonephritis

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.

Download Full-text

SP055DIAGNOSTIC VALUE OF PHOSPHOLIPASE A2 RECEPTOR, IGG4, ALDOSE REDUCTASE AND SUPEROXIDE DISMUTASE IN DETERMINATION OF PRIMARY VERSUS SECONDARY MEMBRANOUS GLOMERULONEPHRITIS

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfv188.18 ◽

2015 ◽

Vol 30 (suppl_3) ◽

pp. iii398-iii398

Author(s):

Kang Wook Lee ◽

Yoo Hyung Kim ◽

Sarah Chung ◽

Dae Eun Choi ◽

Ki Ryang Na ◽

...

Keyword(s):

Superoxide Dismutase ◽

Phospholipase A2 ◽

Aldose Reductase ◽

Membranous Glomerulonephritis ◽

Phospholipase A2 Receptor

Download Full-text

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

SAGE Open Medical Case Reports ◽

10.1177/2050313x19869764 ◽

2019 ◽

Vol 7 ◽

pp. 2050313X1986976 ◽

Cited By ~ 1

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.

Download Full-text

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

10.33118/oaj.clin.2019.01.003 ◽

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.

Download Full-text

Role of phospholipase A2 receptor (PLA2R) antibodies in patients with membranous glomerulonephritis: A prospective study on Indian cohort

10.26226/morressier.578f37fad462b8028d88ff59 ◽

2016 ◽

Author(s):

Afreen Karimkhan

Keyword(s):

Prospective Study ◽

Phospholipase A2 ◽

Membranous Glomerulonephritis ◽

Phospholipase A2 Receptor ◽

A Prospective Study

Download Full-text

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

Case Reports in Nephrology and Dialysis ◽

10.1159/000446019 ◽

2016 ◽

Vol 6 (2) ◽

pp. 76-82 ◽

Cited By ~ 1

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.

Download Full-text