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__NOTOC__
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{{Rapidly progressive glomerulonephritis}}
{{Rapidly progressive glomerulonephritis}}
{{CMG}}
{{CMG}} {{AE}} {{JSS}}


==Overview==
==Overview==
Rapidly progressive glomerulonephritis is classified on the basis of cause of [[Glomerulus|glomerular]] injury.The [[Immunofluorescence|immunoflourescent microspcopic]] findings are used in determining the cause of glomerular injury.


==Classification==
==Classification==
RPGN is classified on the basis of the cause of glomerular injury and the findings from light and immunofluorescence microscopy.<ref name="pmid3287904">{{cite journal| author=Couser WG| title=Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy. | journal=Am J Kidney Dis | year= 1988 | volume= 11 | issue= 6 | pages= 449-64 | pmid=3287904 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3287904  }} </ref><ref name="pmid9507491">{{cite journal| author=Couser WG| title=Pathogenesis of glomerular damage in glomerulonephritis. | journal=Nephrol Dial Transplant | year= 1998 | volume= 13 Suppl 1 | issue=  | pages= 10-5 | pmid=9507491 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9507491  }} </ref>.


===Type I===
===Type I===
Accounting for approximately 20% of RPGN, type I RPGN is characterized by the presence of [[autoantibodies]] directed against the [[glomerular basement membrane]] (GBM). It is also called anti-GBM glomerulonephritis. The antibodies are directed against a particular protein found in the GBM, [[type IV collagen]], specifically the noncollagenous region of its α<sub>3</sub> chain.<ref name="robbins">{{cite book |author=Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. |title=Robbins and Cotran pathologic basis of disease |publisher=Elsevier Saunders |location=St. Louis, MO |year=2005 |pages=pp976-8 |isbn=0-7216-0187-1 |oclc= |doi=}}</ref>
* Type I RPGN is characterized by the presence of [[autoantibodies]] directed against the [[glomerular basement membrane]] (GBM)<ref name="pmid8909258">{{cite journal| author=Heeringa P, Brouwer E, Klok PA, Huitema MG, van den Born J, Weening JJ et al.| title=Autoantibodies to myeloperoxidase aggravate mild anti-glomerular-basement-membrane-mediated glomerular injury in the rat. | journal=Am J Pathol | year= 1996 | volume= 149 | issue= 5 | pages= 1695-706 | pmid=8909258 | doi= | pmc=1865281 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8909258  }} </ref>.  
 
* Type I also known as anti-[[GBM]] [[Glomerular disease|glomerulonephritis.]]
In addition to the anti-GBM antibodies, some cases of type I RPGN are also associated with antibodies directed against the [[basement membrane]] of lung [[alveoli]], producing [[Goodpasture syndrome]]. The majority of type I disease, however, features anti-GBM antibodies alone; these cases are considered idiopathic.<ref name="robbins"/>
* The antibodies formed are known as anticollagen antibodies and react against type IV [[collagen]] of [[GBM]].<ref name="robbins">{{cite book |author=Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. |title=Robbins and Cotran pathologic basis of disease |publisher=Elsevier Saunders |location=St. Louis, MO |year=2005 |pages=pp976-8 |isbn=0-7216-0187-1 |oclc= |doi=}}</ref>
* The antibodies can be produced by a stimulus such as viral [[Upper respiratory tract infection|URTI]] that exposes [[Alveolus|alveolar]] collagen membrane or it can be idiopathic.
* The antibodies formed can act against alveolar membrane and lungs get involved in some cases such as in [[goodpasture syndrome]].


===Type II===
===Type II===
RPGN caused by the deposition of [[immune complex]]es accounts for 25% of RPGN and is classified as type II. Thus any [[immune complex disease]] that involves the glomerulus may progress to RPGN if severe enough. These diseases include [[systemic lupus erythematosus]], [[postinfectious glomerulonephritis]], [[Henoch-Schönlein purpura]], and [[IgA nephropathy]].<ref name="robbins"/>
* Type II RPGN is caused by the deposition of [[Immune complex|immune complexes]] in the [[GBM|GBM.]]
* Immune complexes can be formed in certain infections or in [[Connective tissue disease|connective tissue disorders.]]
* These immune complexes deposit over the GBM and activate the [[complement system]] resulting in [[Crescent Rising|crescent]] formation.
* Examples include:
* Postinfectious ([[Staphylococcus aureus|staphylococci]]/[[Streptococcus|streptococci]])
* [[Connective tissue disease|Connective tissue disorders]]
* [[Lupus nephritis]]
* [[Henoch-Schönlein purpura|Henoch-Schönlein purpura]]
* [[IgA nephropathy|Immunoglobulin A nephropathy]]
* Mixed [[cryoglobulinemia]]
* [[Membranoproliferative glomerulonephritis]]


===Type III===
===Type III===
Also known as pauci-immune RPGN, type III RPGN accounts for 55% of RPGN and features neither immune complex deposition nor anti-GBM antibodies. Instead, the glomeruli are damaged in an undefined manner, perhaps through the activation of [[neutrophil]]s in response to [[anti-neutrophil cytoplasmic antibodies]] (ANCA). Type III RPGN may be isolated to the glomerulus (primary, or idiopathic) or associated with a systemic disease (secondary). In most cases of the latter, the systemic disease is an ANCA-associated [[vasculitis]] such as [[Wegener granulomatosis]], [[microscopic polyangiitis]], or [[Churg-Strauss syndrome]].<ref name="robbins"/>
* Type III RPGN is also known as pauci immune RPGN<ref name="robbins" />.
 
* There are no anti [[GBM]] antibodies or no [[Immune complex|immune complexes]] involved.
Classification of type III RPGN into primary or secondary may be unnecessary, as primary type III RPGN and secondary type III RPGN may represent a spectrum of the same disease process.<ref name="robbins"/>
* It is further classified into 2 types:
** Immunogenic - [[Anti-neutrophil cytoplasmic antibody|ANCA]] positive
** Non immunogenic- ANCA negative/ Idiopathic
* ANCAs cause the release of lytic enzymes from [[Neutrophil|neutrophils]] that damage the GBM.
* Systemic [[vasculitis]] is present in most of the cases but some occur without systemic involvement and only renal findings maybe present.
* Examples include 
** [[Granulomatosis with polyangiitis]] (Wegener granulomatosis)
** [[Microscopic polyangiitis]] (MPA)
** Renal-limited necrotizing crescentic glomerulonephritis (NCGN)
** [[Langerhans cell histiocytosis|Eosinophilic granulomatosis]] with polyangiitis (EGPA; Churg-Strauss syndrome)
** Drugs- [[hydralazine]], [[allopurinol]] and [[rifampin]].


==References==
==References==

Latest revision as of 19:26, 24 July 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2]

Overview

Rapidly progressive glomerulonephritis is classified on the basis of cause of glomerular injury.The immunoflourescent microspcopic findings are used in determining the cause of glomerular injury.

Classification

RPGN is classified on the basis of the cause of glomerular injury and the findings from light and immunofluorescence microscopy.[1][2].

Type I

Type II

Type III

References

  1. ↑ Couser WG (1988). "Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy". Am J Kidney Dis. 11 (6): 449–64. PMID 3287904.
  2. ↑ Couser WG (1998). "Pathogenesis of glomerular damage in glomerulonephritis". Nephrol Dial Transplant. 13 Suppl 1: 10–5. PMID 9507491.
  3. ↑ Heeringa P, Brouwer E, Klok PA, Huitema MG, van den Born J, Weening JJ; et al. (1996). "Autoantibodies to myeloperoxidase aggravate mild anti-glomerular-basement-membrane-mediated glomerular injury in the rat". Am J Pathol. 149 (5): 1695–706. PMC 1865281. PMID 8909258.
  4. ↑ 4.0 4.1 Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, MO: Elsevier Saunders. pp. pp976–8. ISBN 0-7216-0187-1.
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