Nephrotic syndrome caused by membranous nephropathy with solitary IgA deposition along the capillary wall

Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in adults. Subepithelial polyclonal immunoglobulin deposits and >70% M-type phospholipase A2 receptor antibody positivity are typical findings in idiopathic MN. A 58-year-old female patient was admitted with clinical presentation of nephrotic syndrome. Autoimmune diseases, infections, and malignancies were ruled out after clinical and laboratory evaluations. Diagnostic work-up revealed serum PLA2R antibody negativity and diffuse thickening of glomerular capillary wall on biopsy, while glomerular capillary wall IgG, C3, and Lambda monotypic light chain deposition and PLA2R1 positivity were detected by immunofluorescence and immunohistochemical examination, respectively. Following prednisolone treatment, creatinine and proteinuria were markedly regressed. The MN cases with a light chain deposits are rare and experience regarding their treatment are insufficient.

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Membranous Nephropathy in the Dog

Veterinary Pathology ◽

10.1177/030098588602300611 ◽

1986 ◽

Vol 23 (6) ◽

pp. 718-733 ◽

Cited By ~ 20

Author(s):

R. S. Jaenke ◽

T. A. Allen

Keyword(s):

Nephrotic Syndrome ◽

Membranous Nephropathy ◽

Basement Membranes ◽

Capillary Wall ◽

Glomerular Capillary Wall ◽

Renal Lesions ◽

Pathologic Findings ◽

Advanced Stages ◽

Recovery Characteristic ◽

Immunoglobulin Deposits

Membranous nephropathy, a disease syndrome characterized by severe proteinuria and often accompanied by the nephrotic syndrome, was identified in 29% of a population of 46 proteinuric dogs. Renal lesions were characterized by the presence of subepithelial immunoglobulin deposits distributed diffusely along the glomerular capillary wall. Advanced stages were associated with progressive thickening of capillary basement membranes and incorporation of the immune deposits. These changes were followed by either glomerulosclerosis or recovery. Characteristic morphologic stages were correlated with clinical pathologic findings which showed that the level of proteinuria, hypoalbuminemia, and consequent nephrotic syndrome was most severe in the initial stages of membranous nephropathy while the level of azotemia increased in the more advanced stages of the syndrome.

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Nephrotic syndrome secondary to alpha-1 antitrypsin deficiency

BMJ Case Reports ◽

10.1136/bcr-2020-240288 ◽

2021 ◽

Vol 14 (3) ◽

pp. e240288

Author(s):

Gabriela F Santos ◽

Paul Ellis ◽

Daniela Farrugia ◽

Alice M Turner

Keyword(s):

Nephrotic Syndrome ◽

Membranous Nephropathy ◽

Clinical Investigation ◽

Genetic Disorder ◽

Clinical Manifestations ◽

Protective Role ◽

Caucasian Woman ◽

Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin

We report a 64-year-old caucasian woman diagnosed with membranous nephropathy secondary to alpha-1 antitrypsin deficiency (AATD). AATD is a rare autosomal codominant genetic disorder. Its clinical manifestations are mostly observed in the lungs, with early-onset emphysema. Nephropathy due to AATD is still very rare and only a few cohort studies have been reported. It has been recognised that alpha-1 antitrypsin has a protective role in the kidneys which enhances the possibility of development of kidney failure, such as nephrotic syndrome, in cases of AATD. Further clinical investigation is needed to understand the relationship between the development of nephropathy, namely membranous nephropathy, and AATD.

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A CASE OF MEMBRANOUS NEPHROPATHY IN WHICH GASTRECTOMY FOR A GASTRIC CANCER LED TO A REMISSION OF NEPHROTIC SYNDROME

The journal of the Japanese Practical Surgeon Society ◽

10.3919/ringe1963.58.1864 ◽

1997 ◽

Vol 58 (8) ◽

pp. 1864-1868 ◽

Cited By ~ 1

Author(s):

Kentaro EMOTO ◽

Tadateru TAKAHASHI ◽

Yoshitaka KATO ◽

Takayuki NOMIMURA ◽

Ichiro OMORI ◽

...

Keyword(s):

Gastric Cancer ◽

Nephrotic Syndrome ◽

Membranous Nephropathy

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Idiopathic Nephrotic Syndrome: Proliferative Nephropathy and Membranous Nephropathy

Persistent Renal-Genitourinary Disorders ◽

10.1007/978-1-4613-2339-6_1 ◽

1987 ◽

pp. 3-22

Author(s):

Russell W. Chesney ◽

Antonia C. Novello

Keyword(s):

Nephrotic Syndrome ◽

Membranous Nephropathy ◽

Idiopathic Nephrotic Syndrome

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Effect of Prolonged Tacrolimus Treatment in Idiopathic Membranous Nephropathy with Nephrotic Syndrome

Pharmacology ◽

10.1159/000348570 ◽

2013 ◽

Vol 91 (5-6) ◽

pp. 259-266 ◽

Cited By ~ 10

Author(s):

Hang Yuan ◽

Nian Liu ◽

Guang-Dong Sun ◽

Ye Jia ◽

Ping Luo ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Membranous Nephropathy ◽

Idiopathic Membranous Nephropathy

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Immunosuppressive treatment for idiopathic membranous nephropathy in adults with nephrotic syndrome

10.1002/14651858.cd004293 ◽

2003 ◽

Cited By ~ 1

Author(s):

A Schieppati ◽

A Perna ◽

J Zamora ◽

GA Giuliano ◽

N Braun ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Membranous Nephropathy ◽

Immunosuppressive Treatment ◽

Idiopathic Membranous Nephropathy

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Membranous nephropathy

Oxford Textbook of Medicine ◽

10.1093/med/9780199204854.003.210804 ◽

2010 ◽

pp. 3985-3988

Author(s):

Dwomoa Adu

Keyword(s):

Prostate Cancer ◽

Systemic Lupus Erythematosus ◽

Nephrotic Syndrome ◽

Basement Membrane ◽

Autoimmune Diseases ◽

Outer Surface ◽

Glomerular Basement Membrane ◽

Lupus Erythematosus ◽

Membranous Nephropathy ◽

Systemic Lupus

Membranous nephropathy, which accounts for 20 to 30% of cases of the nephrotic syndrome in adults, is defined histologically by the presence of subepithelial immune deposits on the outer surface of the glomerular basement membrane. The immune mechanisms that lead to this are uncertain, and most cases are of unknown cause (idiopathic), but the condition can be associated with autoimmune diseases (systemic lupus erythematosus), malignancy (in 10% of cases, most commonly lung and prostate cancer), drugs, and infections....

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