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==Treatment==
==Treatment==


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


 
==Case Studies==
==Classification==
[[Rapidly progressive glomerulonephritis case study one|Case #1]]
===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>
 
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"/>
 
===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 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"/>
 
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"/>
 


==References==
==References==

Revision as of 13:30, 28 September 2012

Rapidly progressive glomerulonephritis
ICD-10 N00-N08 with .7 suffix
DiseasesDB 3165

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords:: Crescentic glomerulonephritis; RPGN

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