{{Infobox medical condition (new) | name = Rapidly progressive glomerulonephritis | synonyms = Crescentic glomerulonephritis<ref>{{cite web |last1=RESERVED |first1=INSERM US14-- ALL RIGHTS |title=Orphanet: Rapidly progressive glomerulonephritis |url=https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=280569 |access-date=31 July 2019 |website=www.orpha.net |language=en}}</ref> | image = Crescentic glomerulonephritis (2).jpg | caption = Histopathological image of crescentic glomerulonephritis in a patient with MPO-ANCA positive rapid progressive glomerulonephritis. Hematoxylin & eosin stain. | pronounce = | field = Nephrology | | symptoms = Hematuria<ref name="robbins"/> | complications = | onset = | duration = | types = Type I, II and III<ref name=isbn1-4200-8478-X/> | causes = | risks = | diagnosis = Serum analysis<ref name=robbins/> | differential = | prevention = | treatment = Corticosteroids | medication = | prognosis = | frequency = | deaths = }}
'''Rapidly progressive glomerulonephritis''' ('''RPGN''') is a syndrome of the kidney that is characterized by a rapid loss of kidney function,<ref name="McGraw-Hill">[http://medical-dictionary.thefreedictionary.com/rapidly+progressive+glomerulonephritis TheFreeDictionary > rapidly progressive glomerulonephritis] Citing: McGraw-Hill Concise Dictionary of Modern Medicine. 2002</ref><ref name="emedicine">[http://emedicine.medscape.com/article/239504-overview eMedicine > Glomerulonephritis, Crescentic] Author: Malvinder S Parmar. Updated: Sep 25, 2008</ref> (usually a 50% decline in the glomerular filtration rate (GFR) within 3 months)<ref name=emedicine/> with glomerular ''crescent formation'' seen in at least 50%<ref name=emedicine/> or 75%<ref name=McGraw-Hill/> of glomeruli seen on kidney biopsies. If left untreated, it rapidly progresses into acute kidney failure<ref>{{DorlandsDict|four/000045115|rapidly progressive glomerulonephritis}}</ref> and death within months. In 50% of cases, RPGN is associated with an underlying disease such as Goodpasture syndrome, systemic lupus erythematosus or granulomatosis with polyangiitis; the remaining cases are idiopathic. Regardless of the underlying cause, RPGN involves severe injury to the kidneys' glomeruli, with many of the glomeruli containing characteristic glomerular crescents (crescent-shaped scars).<ref name="pmid32132388">{{cite journal |vauthors=Anguiano L, Kain R, Anders HJ |date=May 2020 |title=The glomerular crescent: triggers, evolution, resolution, and implications for therapy |journal=Current Opinion in Nephrology and Hypertension |volume=29 |issue=3 |pages=302–309 |doi=10.1097/MNH.0000000000000596 |pmc=7170443 |pmid=32132388}}</ref>
==Signs and symptoms== Most types of RPGN are characterized by severe and rapid loss of kidney function with marked hematuria; red blood cell casts in the urine; and proteinuria sometimes exceeding three grams in twenty-four hours, a combination of signs associated with nephritic syndrome. Some patients also experience hypertension and edema. Severe disease is characterized by pronounced oliguria or anuria, which portends a poor prognosis.<ref name="robbins"/>
==Pathophysiology== thumb|130 px It is thought that antineutrophil cytoplasmic antibodies (ANCA) interact with antigens in the cytoplasm of neutrophils to cause an early{{citation needed|date=March 2020}} degranulation, triggering the release of lytic enzymes at the site of injury<ref name="anc">{{cite web |date=2018-04-05 |title=Rapidly Progressive Glomerulonephritis: Background, Pathophysiology, Epidemiology |url=http://emedicine.medscape.com/article/240457-overview#a5}}</ref> and leading to the formation of ''glomerular crescents'' that consist primarily of parietal epithelial cells from Bowman's capsule and in some cases podocytes.<ref name="pmid4125698">{{cite journal |vauthors=Morita T, Suzuki Y, Churg J |date=September 1973 |title=Structure and development of the glomerular crescent |journal=The American Journal of Pathology |volume=72 |issue=3 |pages=349–68 |pmc=1904036 |pmid=4125698}}</ref>
==Diagnosis== Serum analysis often aids in the diagnosis of a specific underlying disease. The presence of anti-glomerular basement membrane (GBM) antibodies suggests type I RPGN; antinuclear antibodies (ANA) may support a diagnosis of systemic lupus erythematosus and type II RPGN; and type III and idiopathic RPGN are frequently associated with anti-neutrophil cytoplasmic antibodies (ANCA)-positive serum.<ref name="robbins"/>
Impaired kidney function in an individual who has had the condition for fewer than three months is characteristic of RPGN. An ultrasonographic examination of the abdomen should be obtained. Although the presence of sediment in the urine on examination can indicate proliferative glomerulonephritis, many cases of rapidly progressive glomerulonephritis need a renal biopsy to make a diagnosis.<ref>{{Cite journal |last=Bhowmik |first=Dipankar |date=January 2011 |title=Clinical Approach to Rapidly Progressive Renal Failure |url=http://www.japi.org/january_2011/oa_%20clinical%20approach.pdf |journal=Journal of the Association of Physicians of India |volume=59 |pages=38–41 |pmid=21751663 |access-date=31 October 2015}}</ref>
===Classification=== thumb|Crescentic glomerulonephritis RPGN can be classified into three types, based upon the immunofluorescence patterns:<ref name="isbn1-4200-8478-X">{{cite book |author=Ghosh, Amit K. |title=Mayo Clinic Internal Medicine Review: Eighth Edition (Mayo Clinic Internal Medicine Review) |publisher=Informa Healthcare |year=2008 |isbn=978-1-4200-8478-8 |pages=694}}</ref>
====Type I==== Accounting for approximately 3<ref name="auto">{{Cite web |title=Table:Classification of Rapidly Progressive Glomerulonephritis Based on Immunofluorescence Microscopy |url=https://www.msdmanuals.com/professional/multimedia/table/classification-of-rapidly-progressive-glomerulonephritis-based-on-immunofluorescence-microscopy |website=MSD Manual Professional Edition}}</ref>% of RPGN, type I RPGN, also called anti-GBM glomerulonephritis, is characterized by the presence of autoantibodies directed against type IV collagen (specifically, the noncollagenous region of its α<sub>3</sub> chain)<ref name="robbins">{{cite book |author1=Cotran, Ramzi S. |title=Robbins and Cotran pathologic basis of disease |author2=Kumar, Vinay |author3=Fausto, Nelson |author4=Nelso Fausto |author5=Robbins, Stanley L. |author6=Abbas, Abul K. |publisher=Elsevier Saunders |year=2005 |isbn=978-0-7216-0187-8 |location=St. Louis, MO |pages=976–8}}</ref> in the glomerular basement membrane (GBM). Some cases are 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==== Characterized by deposition of immune complexes in glomerular tissues, type II RPGN accounts for 40<ref name="auto"/>% of cases. Any immune complex disease—including systemic lupus erythematosus, acute proliferative glomerulonephritis, Henoch–Schönlein purpura, and IgA nephropathy—that involves the glomerulus may progress to RPGN if severe enough.<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 neutrophils in response to 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 granulomatosis with polyangiitis, microscopic polyangiitis or eosinophilic granulomatosis with polyangiitis.<ref name="robbins"/>
==Treatment== Therapy consists of a combination of rituximab,<ref name=":0">{{Cite journal |last=Greenhall |first=George H.B. |last2=Salama |first2=Alan D. |date=2015 |title=What is new in the management of rapidly progressive glomerulonephritis? |journal=Clinical Kidney Journal |volume=8 |issue=2 |pages=143–150 |doi=10.1093/ckj/sfv008 |issn=2048-8505 |pmc=4370308 |pmid=25815169}}</ref> corticosteroids, and cyclophosphamide, with a substitution of azathioprine for cyclophosphamide after a ninety-day initial period being another option.<ref name=":0" /> When remission is achieved, immunosuppressants are still used,<ref name=":1">{{Cite journal |last=Jennette |first=J. Charles |last2=Nachman |first2=Patrick H. |date=2017-10-06 |title=ANCA Glomerulonephritis and Vasculitis |url=https://cjasn.asnjournals.org/content/12/10/1680 |journal=Clinical Journal of the American Society of Nephrology |language=en |volume=12 |issue=10 |pages=1680–1691 |doi=10.2215/CJN.02500317 |issn=1555-9041 |pmc=5628710 |pmid=28842398 |doi-access=free}}</ref> usually corticosteroids with azathioprine or rituximab infusions.<ref name=":1" />
==Epidemiology== The incidence rate of rapidly progressive glomerulonephritis is approximately 3.9 individuals per million.<ref>{{Cite journal |last=Hedger |first=Neil |last2=Stevens |first2=Judith |last3=Drey |first3=Nick |last4=Walker |first4=Sarah |last5=Roderick |first5=Paul |date=2000-10-01 |title=Incidence and outcome of pauci‐immune rapidly progressive glomerulonephritis in Wessex, UK: a 10‐year retrospective study |journal=Nephrology Dialysis Transplantation |language=en |volume=15 |issue=10 |pages=1593–1599 |doi=10.1093/ndt/15.10.1593 |issn=0931-0509 |pmid=11007827 |doi-access=free}}</ref>
==References== {{Reflist|32em}}
==Further reading== * {{Cite journal |last=Greenhall |first=George H. B. |last2=Salama |first2=Alan D. |date=2015-02-19 |title=What is new in the management of rapidly progressive glomerulonephritis? |journal=Clinical Kidney Journal |language=en |volume=8 |issue=2 |pages=143–50 |doi=10.1093/ckj/sfv008 |issn=2048-8505 |pmc=4370308 |pmid=25815169}} == External links == {{Medical resources | DiseasesDB = 3165 | ICD10 = {{ICD10|N01}} | ICD9 = | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = med | eMedicineTopic = 881 | eMedicine_mult = {{eMedicine2|med|890}} | MeshID = | Orphanet = 280569 }} {{Commons}}
{{Scholia|topic}} {{Glomerular disease}}
Category:Kidney diseases