scholarly journals Immune Complex Membranoproliferative Glomerulonephritis Associated with Transjugular Intrahepatic Portosystemic Shunts in Alcoholic Cirrhosis: Two Case Reports

2017 ◽  
Vol 26 (3) ◽  
pp. 286-288 ◽  
Author(s):  
Julio Hernández-Jaras ◽  
Jordi Espí-Reig ◽  
Rafael Alis ◽  
Ana-Maria García-Martínez ◽  
Diego Rodríguez-Ortega ◽  
...  
Keyword(s):  
Immune Complex ◽  
Membranoproliferative Glomerulonephritis ◽  
Case Reports ◽  
Alcoholic Cirrhosis ◽  
Portosystemic Shunts

Morphofunctional Effects of C5 Convertase Blockade in Immune Complex-Mediated Membranoproliferative Glomerulonephritis: Report of Two Cases with Evidence of Terminal Complement Activation

Nephron ◽  
2020 ◽  
Vol 144 (4) ◽  
pp. 195-203
Author(s):  
Camillo Carrara ◽  
Manuel Alfredo Podestà ◽  
Mauro Abbate ◽  
Paola Rizzo ◽  
Rossella Piras ◽  
...  
Keyword(s):  
Immune Complex ◽  
Complement Activation ◽  
Membranoproliferative Glomerulonephritis ◽  
Terminal Complement

Therapy and outcomes of C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis

2020 ◽  
Author(s):  
Priyanka Khandelwal ◽  
Swati Bhardwaj ◽  
Geetika Singh ◽  
Aditi Sinha ◽  
Pankaj Hari ◽  
...  
Keyword(s):  
Immune Complex ◽  
Membranoproliferative Glomerulonephritis ◽  
C3 Glomerulopathy

scholarly journals Demographic, clinical characteristics and treatment outcomes of immune-complex membranoproliferative glomerulonephritis and C3 glomerulonephritis in Japan: A retrospective analysis of data from the Japan Renal Biopsy Registry

PLoS ONE ◽  
2021 ◽  
Vol 16 (9) ◽  
pp. e0257397
Author(s):  
Naoki Nakagawa ◽  
Masashi Mizuno ◽  
Sawako Kato ◽  
Shoichi Maruyama ◽  
Hiroshi Sato ◽  
...  
Keyword(s):  
Renal Function ◽  
Renal Biopsy ◽  
Treatment Outcomes ◽  
Immune Complex ◽  
Membranoproliferative Glomerulonephritis ◽  
Clinical Characteristics ◽  
Diastolic Pressure ◽  
Remission Rate ◽  
Type I ◽  
C3 Glomerulonephritis

The reclassification of membranoproliferative glomerulonephritis (MPGN) into immune-complex MPGN (IC-MPGN) and C3 glomerulopathy (C3G) based on immunofluorescence findings in kidney biopsies has provided insights into these two distinct diseases. C3G is further classified into dense deposit disease and C3 glomerulonephritis (C3GN) based on electron micrographic findings. Although these diseases have poor outcomes, limited Japanese literature confined to small, single-center cohorts exist on these diseases. We retrospectively analyzed 81 patients with MPGN type I and III from 15 hospitals in the Japan Renal Biopsy Registry to compare demographic, clinical characteristics and treatment outcomes of patients with IC-MPGN to those with C3GN. Of the 81 patients reviewed by immunofluorescence findings in kidney biopsies, 67 patients had IC-MPGN and 14 patients had C3GN. Age at diagnosis and systolic and diastolic pressure were higher and proteinuria and impaired renal function were significantly more prevalent in patients with IC-MPGN than those with C3GN. About 80% of the patients in both groups were treated with immunosuppressive therapy. At last follow-up (median 4.8 years), complete remission rate of proteinuria was significantly higher in patients with C3GN (64.3%) than in those with IC-MPGN (29.9%; P = 0.015). The renal survival rate was lower in patients with IC-MPGN when compared to C3GN (73.1% vs. 100%; log-rank, P = 0.031). Systolic blood pressure and renal function at baseline were independent predictors of progression to end-stage kidney disease. The overall prognosis of patients with C3GN is more favorable than for patients with IC-MPGN.

scholarly journals CFHR5 Genetic Variations and Serum Levels in Patients with Immune-Complex-Mediated Membranoproliferative Glomerulonephritis and C3-Glomerulopathy

2020 ◽  
Author(s):  
Nóra Garam ◽  
Marcell Cserhalmi ◽  
Zoltán Prohászka ◽  
Ágnes Szilágyi ◽  
Nóra Veszeli ◽  
...  
Keyword(s):  
Immune Complex ◽  
Membranoproliferative Glomerulonephritis ◽  
Kidney Diseases ◽  
Alternative Pathway ◽  
Serum Levels ◽  
Genetic Alterations ◽  
Factor H ◽  
Complement Factor ◽  
C3 Glomerulopathy

Abstract Background: Factor H-related-5 (FHR-5) is a member of the complement Factor H protein family. Due to the homology to Factor H, the main complement regulator of the alternative pathway, it may also be implicated in pathomechanism of kidney diseases where Factor H and alternative pathway dysregulation play a role. Here, we report the first observational study on CFHR5 variations along with serum FHR-5 levels in immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G) patients together with the clinical, genetic, complement and follow-up data.Results: 120 patients with a histologically-proven diagnosis of IC-MPGN/C3G were enrolled in the study. FHR-5 serum levels were measured in ELISA, the CFHR5 gene was analyzed by Sanger-sequencing, and selected mutants were studied as recombinant proteins in ELISA and SPR.Eight relevant CFHR5 variations in 14 patients (11.7%) were observed, 5 of them identified as pathogenic for C3G. The FHR-5G278S and FHR-5R356H mutations altered the interaction of FHR-5 with C3b, when compared to the FHR-5WT. Serum FHR-5 levels were lower in patients compared to controls. Low serum FHR-5 concentration at presentation associated with better renal survival during the follow-up period, furthermore, it showed clear association with signs of hypocomplementemia and clinically meaningful clusters.Conclusions: Our observations support the hypothesis that FHR-5 protein and its genetic alterations play a role in the pathogenesis of IC-MPGN/C3G.

scholarly journals A case of nephrotic syndrome secondary to HIV immune complex kidney disease

2020 ◽  
Vol 10 (1) ◽  
pp. e09-e09
Author(s):  
Michael Edwards ◽  
Patrick Linden ◽  
Anindya Banerjee
Keyword(s):  
Immune Complex ◽  
Membranoproliferative Glomerulonephritis ◽  
Diabetes Mellitus Type 2 ◽  
Filtration Rate ◽  
Complex Disease ◽  
Estimated Glomerular Filtration Rate ◽  
Treatment Options ◽  
Hiv Positive ◽  
Immune Complex Disease

HIV immune complex disease of the kidney (HIVICK) is a rare but increasingly well-recognised cause of renal dysfunction and proteinuria in HIV-positive patients. A 56-year-old man with known HIV, diabetes mellitus type 2, liver cirrhosis and previous Hepatitis C virus (HCV) presented with a labile estimated glomerular filtration rate and significant proteinuria. Electron microscopy from a renal biopsy identified capillary wall deposition for IgG, IgM, Kappa, Lambda and focal C1q consistent with membranoproliferative glomerulonephritis (MPGN) and associated immune complex disease. A second opinion of the images confirmed the diagnosis of HIVICK. The increased recognition of HIVICK in HIV patients should prompt further research into the causes and treatment options available.

Membranoproliferative glomerulonephritis

2020 ◽  
pp. 4937-4943
Author(s):  
Tabitha Turner-Stokes ◽  
Mark A. Little
Keyword(s):  
Immune Complex ◽  
Membranoproliferative Glomerulonephritis ◽  
Enzyme Inhibitor ◽  
Alternative Pathway ◽  
Rapidly Progressive Glomerulonephritis ◽  
Pressure Control ◽  
Underlying Disease ◽  
Plasma Cell Dyscrasia ◽  
Angiotensin Ii Receptor ◽  
C3 Nephritic Factor

The key histological features of membranoproliferative glomerulonephritis (MPGN) are mesangial hypercellularity, endocapillary proliferation, and capillary wall remodelling. There are two main types: (1) immune complex-mediated disease—caused by chronic infection causing persistent antigenaemia (notably hepatitis C), autoimmune disease, or monoclonal immunoglobulin production by plasma cell dyscrasia, and a few ‘idiopathic’ cases; and (2) complement-mediated disease—caused by dysregulation of the alternative pathway of complement, including by C3 nephritic factor (C3Nef), an autoantibody that stabilizes the alternative pathway C3 convertase. Clinical presentation is varied, including nephrotic syndrome, episodic visible haematuria, hypertension/rapidly progressive glomerulonephritis, asymptomatic nonvisible haematuria, and chronic kidney disease. Treatment depends on the underlying disease. All patients should receive appropriate conservative measures (blood pressure control, angiotensin-converting enzyme inhibitor/angiotensin II receptor blocker). Underlying infection or monoclonal gammopathy should be treated, when possible, in those with immune complex-mediated MPGN. Eculizumab may have a role in treatment of some patients with complement-mediated MPGN. Steroids and cyclophosphamide or mycophenolate mofetil are used in patients with severe idiopathic MPGN.

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