scholarly journals Membranous Nephropathy in the Dog

1986 ◽  
Vol 23 (6) ◽  
pp. 718-733 ◽  
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.

Membranous Nephropathy with Lambda Light Chain Restriction: A Rare Form with Serum Negative and Tissue Positive PLA2R Ab

Nephron ◽  
2021 ◽  
pp. 1-4
Author(s):  
Emel Isiktas Sayilar ◽  
Saba Kiremitci ◽  
Ihsan Ergun ◽  
Arzu Ensari
Keyword(s):  
Nephrotic Syndrome ◽  
Light Chain ◽  
Membranous Nephropathy ◽  
Capillary Wall ◽  
Glomerular Capillary Wall ◽  
Glomerular Capillary ◽  
Phospholipase A2 Receptor ◽  
Antibody Positivity ◽  
Immunoglobulin Deposits ◽  
Light Chain Deposition

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.

Very Late Activation-3 Integrin Is the Dominant β1-Integrin on the Glomerular Capillary Wall: An Immunofluorescence Study in Nephrotic Syndrome

Nephron ◽  
1992 ◽  
Vol 62 (4) ◽  
pp. 382-388 ◽  
Author(s):  
Alberto Baraldi ◽  
Luciana Furci ◽  
Giovanna Zambruno ◽  
Elisabetta Rubbiani ◽  
Giorgio Annessi ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Capillary Wall ◽  
Immunofluorescence Study ◽  
Glomerular Capillary Wall ◽  
Glomerular Capillary

scholarly journals Treatment-resistant nephrotic syndrome in dense deposit disease: complement-mediated glomerular capillary wall injury?

2020 ◽  
Vol 35 (9) ◽  
pp. 1791-1795
Author(s):  
Caroline Duineveld ◽  
Eric J. Steenbergen ◽  
Andrew S. Bomback ◽  
Nicole C. A. J. van de Kar ◽  
Jack F. M. Wetzels
Keyword(s):  
Nephrotic Syndrome ◽  
Capillary Wall ◽  
Dense Deposit Disease ◽  
Treatment Resistant ◽  
Glomerular Capillary Wall ◽  
Glomerular Capillary ◽  
Dense Deposit ◽  
Wall Injury

scholarly journals The Efficacy of Rituximab in the Treatment of Membranous Nephropathy

2021 ◽  
Vol 18 (4) ◽  
pp. 580-587
Author(s):  
Ruby Maharjan ◽  
Jin Wen Wang ◽  
Indra Kumar Shrestha
Keyword(s):  
Monoclonal Antibody ◽  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Retrospective Studies ◽  
Initial Therapy ◽  
B Cell Depletion ◽  
Efficacy And Safety ◽  
Considerable Evidence ◽  
Glomerular Capillary Wall ◽  
Subepithelial Layer

Rituximab is a chimeric monoclonal antibody directed against the CD20 expressed on B cells, originally used to treat lymphoma but is increasingly used for the treatment of autoimmune diseases. Membranous nephropathy is an autoimmune disease resulting from the deposition of IgG and complements components onto the subepithelial layer of the glomerular capillary wall and remains the leading cause of nephrotic syndrome in adults. Several prospective and retrospective studies showed rituximab induces remission and may decrease proteinuria in patients with membranous nephropathy. Considerable evidence supports the use of B-cell depletion as initial therapy in nephrotic patients with membranous nephropathy. This review focuses on the efficacy and safety of rituximab in the treatment of membranous nephropathy.Keywords: Membranous nephropathy; rituximab; treatment

Chronic Graft-Versus-Host Disease-Related Membranous Nephropathy: Report of 3 Cases.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5274-5274
Author(s):  
Kanger Zhu ◽  
Wei-jian Zhu ◽  
Tao Zhang
Keyword(s):  
Nephrotic Syndrome ◽  
Graft Versus Host Disease ◽  
Membranous Nephropathy ◽  
Clinical Manifestations ◽  
Basement Membranes ◽  
Hematopoietic Stem ◽  
Gradual Improvement ◽  
Host Disease ◽  
Graft Versus Host ◽  
Nephritic Syndrome

Abstract Objective: To investigate the clinical manifestation, pathological features, management and prognosis of chronic graft-versus-host disease (cGVHD)-related membranous nephropathy following allogeneic hematopoietic stem cell transplantation (allo-HSCT). Methods: Clinical and pathological data from 3 patients with nephrotic syndrome following allo-HSCT were retrospectively analyzed. Results: cGVHD-related membranous nephropathy occurred in 3 out of 150 patients (2%) undergoing allo-HSCT in our BMT unit. 3 patients, with two male and one female, median age of 15 years, and median interval of 11 months between diagnosis and post-transplantation, typically presented with full nephrotic syndrome, including heavy proteinuria (median 8.1 g/24h), edema, hypoalbuminemia (median 29.6 g/L), and hypercholesterolemia (median 11.7 mmolL). A major finding of renal biopsy that was consistent with membranous nephropathy in early stage was diffusely thickened glomerular basement membranes (GBMs) with deposits of IgG along it. All patients showed evidence of cGVHD. Two patients responded well to steroid, with gradual improvement of clinical symptoms and disappearance of proteinuria, and are still alive without evidence of relapse. But the other one died of disseminated hemorrhagic varicella one month following immunosuppressive therapy. Conclusion: These findings suggest that the kidney may be the target organ of alloimmunity, and membranous nephropathy may be one of clinical manifestations of cGVHD, which is response to steroid, resulting in disappearance of proteinuria. It is necessary for transplant physicians to keep in mind the possibility of cGVHD-related membranous nephropathy when a case of nephritic syndrome is encountered.

Characteristics of mutant mice (ICGN) with spontaneous renal lesions: a new model for human nephrotic syndrome

1989 ◽  
Vol 23 (2) ◽  
pp. 169-174 ◽  
Author(s):  
A. Ogura ◽  
T. Asano ◽  
J. Matsuda ◽  
K. Takano ◽  
M. Nakagawa ◽  
...  
Keyword(s):  
Animal Model ◽  
Nephrotic Syndrome ◽  
Mutant Strain ◽  
Human Disease ◽  
Cellular Proliferation ◽  
Basement Membranes ◽  
Immunofluorescence Technique ◽  
High Concentration ◽  
Renal Lesions ◽  
Glomerular Lesions

Spontaneous nephrotic mice (ICGN mice), a new mutant strain of mouse from outbred ICR, were clinically, macroscopically, histologically and immunohistochemically studied to establish their value as a model for human nephrotic syndrome. Most of the affected mice developed proteinuria, hypoproteinaemia and hypercholesterolaemia, and some of them developed systemic oedema. A high concentration of blood urea nitrogen (BUN) and a low haematocrit value were also observed. The kidneys of severe cases showed a decrease in size and had a yellowish granular surface. These findings indicated that the mice were terminally affected by chronic renal in-sufficiency. Histopathology demonstrated glomerular lesions consisting of thickened basement membranes of the capillary loops with irregular spike-like protrusions and enlargement of the mesangium unaccompanied by cellular proliferation. The immunofluorescence technique revealed positive granular staining for IgA, IgG and IgM and to a lesser extent for C3 along the capillary loops in affected mice. The similarity between this spontaneous disease and human nephrotic syndrome caused by idiopathic glomerular lesions is discussed. ICGN mice may be a useful animal model for this human disease.

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