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Immunoglobulin-Mediated Membranoproliferative Gomerulonephritis
Ig-Mediated Membranoproliferative gGomerulonephritis

Presentation

A degree of azotemia is present in 50 % of cases, but it is the patients presenting with the nephritic syndrome who often have significantly reduced GFR. [lecturio.com]

We report a case in which a patient presented with hypertension, seizures, proteinuria, renal impairment and immune complex-mediated MPGN on kidney biopsy. Months later, she presented with classical TMA. [casereports.bmj.com]

On EM, no immune deposits present. [quizlet.com]

All 3 patients presented with proteinuria, and 2 patients also had hematuria. Kidney function was preserved in 2 patients, whereas 1 patient presented with declining kidney function. [mayoclinic.pure.elsevier.com]

How changes in complement regulation leads to very diverse presentations might be related to the underlying defect. [utswim.wordpress.com]

Workup

Summary of workup of patients with MPGN. Clinical Characteristics of Hepatitis-Negative MPGN with Monoclonal Igs There were 12 women and 16 men with monoclonal/biclonal Igs on electrophoresis and immunofixation studies. [cjasn.asnjournals.org]

Treatment

1 Successful entecavir plus prednisolone treatment for hepatitis B virus-associated membranoproliferative glomerulonephritis: A case report. ( 30633192 ) 2019 2 Characteristics of membranoproliferative glomerulonephritis based on a new classification [malacards.org]

Treatment should then be based on the underlying pathogenesis of MPGN. Recent retrospective studies have tried to compare the course and prognosis between the two groups of MPGN. [kidneynews.org]

In addition, we discuss the diagnosis, treatment, and prognosis of the C3 glomerulopathies. [nature.com]

Treatment: at this advanced stage, transplant and/or dialysis is needed. [quizlet.com]

Therapy of Membranoproliferative Glomerulonephritis (MPGN) The treatment of type 1 MPGN or cases of secondary MPGN is with the treatment of the underlying cause, such as treatment of associated infection (e.g., hepatitis C), autoimmune disorder (e.g., [lecturio.com]

Prognosis

Progression and Prognosis of MPGN The rule of thirds is a useful way to remember the prognosis of MPGN. About one-third of patients undergo spontaneous remission. [lecturio.com]

Prognosis is generally poor. Treatment, when indicated, is with corticosteroids and antiplatelet drugs. [merckmanuals.com]

Primary glomerular diseases: clinical features, treatment and prognosis Table I. [clinicaladvisor.com]

Course: DDD has poor prognosis, with 70% progressing to ESRD in 10 years. It is common for the disease to recur even after transplantation. Prognosis and progression with C3 glomerulonephritis is unknown. [quizlet.com]

Etiology

Describe the epidemiology, etiology, pathogenesis, course, and treatment of immunoglobulin-mediated MPGN and the C3 glomerulopathies (Dense Deposit Disease and C3 glomerulonephritis ) Etiology is in disorders of the alternative pathway of complement activation [quizlet.com]

“Membranoproliferative Glomerulonephritis” Image created by Lecturio Etiology of Membranoproliferative Glomerulonephritis If the etiology of membranoproliferative glomerulonephritis is unknown, then it is called primary or idiopathic MPGN. [lecturio.com]

BACKGROUND: A recent classification of membranoproliferative glomerulonephritis (MPGN) utilizes the presence of immunoglobulin and complements to simplify diagnosis and point towards disease etiology. [ncbi.nlm.nih.gov]

C3/Ig positive immunohistochemical results manifested in more than half of each MPGN etiological group. [hippokratia.gr]

C3 glomerulonephritis is similar in etiology to dense deposit disease, arising as a result of alternate complement activation or mutations in the complement-regulating proteins. C3NeF is commonly present. [emedicine.medscape.com]

Epidemiology

Describe the epidemiology, etiology, pathogenesis, diagnosis, and treatment of post-infectious glomerulonephritis Epidemiology: Most common cause of acute nephritic syndrome. Peak incidence ages 5-12 years, >60 years. [quizlet.com]

Survival of patients with marginal zone lymphoma: analysis of the Surveillance, Epidemiology, and End Results database. Cancer. 2013; 119 (3): 629-38[ DOI ][ PubMed ] 9. Arcaini L, Lazzarino M, Colombo N, Burcheri S, Boveri E, Paulli M, et al. [numonthly.com]

Epidemiology – Indian scenario 7. [slideshare.net]

Eduardo Liceaga” (HGM), categorising it according to the most recent classification and identifying the possible causes, thus determining certain epidemiological and clinical characteristics. [elsevier.es]

Epidemiology of Membranoproliferative Glomerulonephritis (MPGN) MPGN is a rare glomerular disease, and seems to be decreasing in prevalence. [lecturio.com]

Pathophysiology

However, a new Mayo classification of MPGN has recently been proposed that is based on the pathophysiology of MPGN ( 1, 2 ). [kidneynews.org]

Only 2 (9%) of these patients (4 eyes) had retinal pathologic features that potentially could be related to the same underlying pathophysiologic process as their renal disease, and neither had vision loss. [ncbi.nlm.nih.gov]

Recent data suggested that these diseases share a common pathophysiology and that patients can transition between glomerulopathies in this spectrum. [casereports.bmj.com]

[…] et al. [ 1 ] describe their long-term experience in diagnosing membranoproliferative glomerulonephritis (MPGN) and conclude that it is time to abandon the ‘old’ classification (MPGN type I, II, III) and to start with a new classification based on new pathophysiological [link.springer.com]

References: [3] [4] [5] [6] [7] [8] [1] [9] [10] [11] Pathophysiology Structural damage of glomerular filtration barrier → massive renal loss of protein → reactively increased hepatic protein synthesis Loss of negative charge of GBM → loss of selectivity [amboss.com]

Prevention

Prevention of primary manifestations: In individuals with pathogenic mutations in CFH, plasma replacement therapy can control complement activation and prevent ESRD. Surveillance: Periodic eye examinations including funduscopic examination. [slideshare.net]

It may also prevent the action of decay accelerators, including CFH. [path.upmc.edu]

C3 nephritic factor (C3 NeF) Genotyping for certain complement mutations (common in children) Circulating C3 nephritic factor is an IgG autoantibody that directly stabilizes the C3-convertase activating complex of the alternative pathway and thereby prevents [utswim.wordpress.com]

The twin aims of treating membranous nephropathy are first to induce a remission of the nephrotic syndrome and second to prevent the development of end-stage kidney failure. [en.wikipedia.org]

Prevention Prevention of glomerulonephritis is best accomplished by avoiding upper respiratory infections, as well as other acute and chronic infections, especially those of a streptococcal origin. [medical-dictionary.thefreedictionary.com]

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