Membranous glomerulonephritis: Difference between revisions

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   Caption        = Membranous Glomerulonephritis: Electron micrography. An excellent example to show thickened basement membrane and immune complexes. <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small> |
   Caption        = Membranous Glomerulonephritis: Electron micrography. An excellent example to show thickened basement membrane and immune complexes. <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small> |
   DiseasesDB    = 7970 |
   DiseasesDB    = 7970 |
   ICD10          = N03.2 |
   ICD10          = |
   ICD9          = {{ICD9|583.1}} |
   ICD9          = {{ICD9|583.1}} |
   ICDO          = |
   ICDO          = |
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{{Membranous glomerulonephritis}}
{{Membranous glomerulonephritis}}
{{CMG}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{SAH}} {{CZ}} {{HK}} {{JSS}}


'''Associate Editor-In-Chief:'''  {{CZ}}
{{SK}} Extramembranous glomerulopathy; Heymann nephritis; idiopathic membranous glomerulonephritis; idiopathic membranous nephropathy; membranous glomerulonephropathy; membranous glomerulopathy; membranous nephropathy; nephropathy, membranous


==[[Membranous glomerulonephritis overview|Overview]]==


==[[Membranous glomerulonephritis historical perspective|Historical Perspective]]==


'''Membranous glomerulonephritis''' ('''MGN'''), also known as '''membranous nephropathy''', is a slowly progressive disease of the [[kidney]] affecting mostly patients between ages of 30 and 50 years. 85% of MGN cases are classified as ''primary membranous glomerulonephritis'' -- that is to say, the cause of the disease is idiopathic (unknown). This can also be referred to as ''idiopathic membranous nephropathy''.  The remainder is secondary due to :
==[[Membranous glomerulonephritis classification|Classification]]==


* '''autoimmune conditions''' (e.g., systemic lupus erythematosus)
==[[Membranous glomerulonephritis pathophysiology|Pathophysiology]]==
* '''infections''' (e.g., syphilis, malaria, hepatitis B)
* '''drugs''' (e.g., captopril, NSAIDs)
* '''inorganic salts'''
* '''malignant tumors''' (in particular, carcinoma of the lung and colon, and melanoma)


The word ''membranous'' refers to the [[Glomerular_basement_membrane|glomerular basement membrane]] of the kidney; ''glomerulonephritis'' means "a kidney disease affecting the capillaries of the [[Glomerulus|glomeruli]]"; ''[[nephropathy]]'' is a generic term that just refers to any disease of the kidney.
==[[Membranous glomerulonephritis causes|Causes]]==


==[[Pathogenesis]]==
==[[Membranous glomerulonephritis differential diagnosis|Differentiating Membranous glomerulonephritis from other Diseases]]==
MGN is caused by circulating [[immune complex]]. Current research indicates that the majority of the immune complexes are formed via binding of antibodies to antigens ''in situ'' to the glomerular basement membrane. The said antigens may be [[endogenous]] to (from) the basement membrane, or "planted" from systemic circulation.


The immune complex serves as an activator that triggers a response from the C5b - C9 complements, which form a [[Complement_membrane_attack_complex|membrane attack complex]] (MAC) on the glomerular epithelial cells. This, in turn, stimulates release of proteases and oxidants by the mesangial and epithelial cells, damaging the capillary walls and causing them to become "leaky". In addition, the epithelial cells also seem to secrete an unknown mediator that reduces nephrin synthesis and distribution.
==[[Membranous glomerulonephritis epidemiology and demographics|Epidemiology and Demographics]]==


==[[Morphology_(biology)|Morphology]]==
==[[Membranous glomerulonephritis risk factors|Risk Factors]]==
The defining point of MGN is the presence of subepithelial immunoglobulin-containing deposits along the [[glomerular basement membrane]] (GBM). By light microscopy, the basement membrane is observed to be diffusively thickened. Using [[Jones' stain]], the GBM appears to have a "spiked" or "holey" appearance.  On electron microscopy, subepithelial deposits that nestle against the glomerular basement membrane seems to be the cause of the thickening. Also, the podocytes lose their foot processes.


As the disease progresses, the deposits will eventually be cleared, leaving cavities in the basement membrane. These cavities will later be filled with basement membrane-like material, and if the disease continues even further, the glomeruli will become sclerosed and finally hyalinized.
==[[Membranous glomerulonephritis screening|Screening]]==
 
Immunoflourescence microscopy will reveal typical granular deposition of immunoglobulins and complement along the basement membrane.
 
== Clinical Presentation ==
Some patients may present as [[nephrotic syndrome]] with [[proteinuria]], [[edema]] with or without [[renal failure]]. Others may be asymptomatic and may be picked up on screening or [[urinalysis]] as having [[proteinuria]]. A definitive diagnosis of membranous nephropathy requires a kidney [[biopsy]].


==[[Membranous glomerulonephritis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


==Diagnosis==
[[Membranous glomerulonephritis history and symptoms|History and Symptoms]] | [[Membranous glomerulonephritis physical examination|Physical Examination]] | [[Membranous glomerulonephritis laboratory findings|Laboratory Findings]] | [[Membranous glomerulonephritis renal biopsy|Renal Biopsy]] | [[Membranous glomerulonephritis light microscopy|Light Microscopy]] | [[Membranous glomerulonephritis CT|CT]] | [[Membranous glomerulonephritis immunofluorescence microscopy|Immunofluorescence Microscopy]] | [[Membranous glomerulonephritis ultrasound|Ultrasound]] | [[Membranous glomerulonephritis other imaging findings|Other Imaging Findings]] | [[Membranous glomerulonephritis other diagnostic studies|Other Diagnostic Studies]]


==Treatment==
==Treatment==
[[Membranous glomerulonephritis medical therapy|Medical Therapy]] | [[Membranous glomerulonephritis surgery|Surgery]] | [[Membranous glomerulonephritis primary prevention|Primary Prevention]] | [[Membranous glomerulonephritis secondary prevention|Secondary Prevention]] | [[Membranous glomerulonephritis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] |  [[Membranous glomerulonephritis future or investigational therapies|Future or Investigational Therapies]]


Treatment of secondary membranous nephropathy is guided by the treatment of the original disease. For treatment of idiopathic membranous nephropathy, the treatment options include [[immunosuppressive]] drugs and non-specific anti-proteinuric measures.
==Case Studies==
===[[Immunosuppressive]] therapy===
# [[Corticosteroids]]: They have been tried with mixed results, with one study showing prevention of progression to [[renal failure]] without improvement in [[proteinuria]].
# [[Chlorambucil]]
# [[Cyclosporine]]
# [[Tacrolimus]]
# [[Cyclophosphamide]]
# [[Mycophenolate mofetil]]
 
Perhaps the most difficult aspect of membranous glomerulonpehritis is deciding which patients to treat with immunosuppressive therapy as opposed to simple "background" or anti-proteinuric therapies.  A large part of this difficulty is due to a lack of ability to predict which patient will progress to end-stage renal disease, or renal disease severe enough to require dialysis.  Because the above medications carry risk, treatment should not be initiated without careful consideration as to risk/benefit profile.  Of note, corticosteroids (typically Prednisone) alone are of little benefit.  They should be combined with one of the other 5 medications, each of which, along with prednisone, has shown some benefit in slowing down progression of membranous nephropathy.  It must be kept in mind, however, that each of the 5 medications also carry their own risks, on top of prednisone.
 
==Natural History==
About a third of patients have spontaneous remission, another third progress to require [[dialysis]] and the last third continue to have proteinuria, without progression of [[renal failure]].
 
==Pathological Findings==
<small> [http://www.peir.net Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>
<br>
 
<div align="center">
<gallery heights="175" widths="125">
image:Membranous GN.jpg|Membranous Glomerulonephritis: Electron micrography. An excellent example to show thickened basement membrane and immune complexes.
image:Membranous GN 1.jpg|Membranous Glomerulonephritis: Micro trichrome high mag excellent to show thickened capillary basement membranes
image:Membranous GN 2.jpg|Membranous Glomerulonephritis: Micro PAS high mag excellent example of this lesion
image:Membranous GN 3.jpg|Membranous Glomerulonephritis: Micro PAS med mag
</gallery>
</div>


==References==
[[Membranous glomerulonephritis case study one|Case #1]]
{{reflist|2}}
<br>


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Latest revision as of 06:34, 28 July 2020

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Membranous glomerulonephritis
Membranous Glomerulonephritis: Electron micrography. An excellent example to show thickened basement membrane and immune complexes.
Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology
ICD-9 583.1
DiseasesDB 7970
MeSH D015433

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Syed Ahsan Hussain, M.D.[2] Cafer Zorkun, M.D., Ph.D. [3] Syed Hassan A. Kazmi BSc, MD [4] Jogeet Singh Sekhon, M.D. [5]

Synonyms and keywords: Extramembranous glomerulopathy; Heymann nephritis; idiopathic membranous glomerulonephritis; idiopathic membranous nephropathy; membranous glomerulonephropathy; membranous glomerulopathy; membranous nephropathy; nephropathy, membranous

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Membranous glomerulonephritis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

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Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Renal Biopsy | Light Microscopy | CT | Immunofluorescence Microscopy | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

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