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{{Infobox_Disease
| Name          = {{PAGENAME}}
| Image          =
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| DiseasesDB    = 3165
| ICD10          = N00-N08 with .7 suffix
| ICD9          =
| ICDO          =
| OMIM          =
| MedlinePlus    =
| eMedicine_mult = {{eMedicine2|med|890}} |
| MeshID        =
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{{Rapidly progressive glomerulonephritis}}
{{Rapidly progressive glomerulonephritis}}
'''For patient information, click [[Rapidly progressive glomerulonephritis (patient information)|here]]'''


'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
{{CMG}}; {{AE}} {{AEL}} {{JSS}} {{SAH}}


{{CMG}}
{{SK}} Acute cresent glomerulonephritis, RPGN


{{SK}}: Crescentic glomerulonephritis; RPGN
==[[Rapidly progressive glomerulonephritis overview|Overview]]==
==[[Rapidly progressive glomerulonephritis overview|Overview]]==
Rapidly progressive glomerulonephritis (RPGN) is one of the few nephrological emergency. However, it fortunately only affects 1-4% of all cases of glomerulonephritis. It is a clinical syndrome that includes signs and symptoms of glomerulonephritis, including hematuria, proteinuria, and edema with signs of renal failure and diffuse crescent formation on histopathology. Without appropriate treatment, RPGN progresses into end-stage renal disease within several days to only a few months yielding a very poor prognosis and renal outcome. RPGN is classified based on the presence of absence of anti-neutrophil cytoplasmic antibody (ANCA) and anti-GBM antibodies. Due to its rarity, the pathogenesis of RPGN is poorly understood and most probably is related to the type of RPGN and the circulating antibodies associated with each type. Evidence on treatment options for RPGN is poor; but the use of glucocorticoids and cyclophosphamide is currently recommended. Basic research and clinical data are currently emerging to better understand the disease pathogenesis and optimal therapeutic options.
 
==[[Rapidly progressive glomerulonephritis historical perspective|Historical Perspective]]==


==[[Rapidly progressive glomerulonephritis classification|Classification]]==
==[[Rapidly progressive glomerulonephritis classification|Classification]]==
===Old Classification===
Following its initial description in 1914, crescenteric glomerulonephritis was first classified by Couser in 1988 based on features of immunofluorescence.<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=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3287904 }} </ref>
====Type I: Anti-GBM Glomerulonephritis====
20% of patients
Presence of linear staining of glomerular basement membrane (GBM)
====Type II: Pauci-Immune Glomerulonephritis====
50% of patients
Absent or minimal immune deposits
====Type III: Immune Complex-Mediated Glomerulonephritis====
30% of patients
Presence of granular patterns of immune deposits within the glomerulus. Immune deposition may be associated with any of the following conditions:
*Infections
*Systemic illnesses
*Other primary glomerular diseases
===New Classification===
Upon the detection of new serological markers such as anti-GBM antibodies and anti-neutrophil cytoplasmic antibodies (ANCA)<ref name="pmid9744974">{{cite journal| author=Hricik DE, Chung-Park M, Sedor JR| title=Glomerulonephritis. | journal=N Engl J Med | year= 1998 | volume= 339 | issue= 13 | pages= 888-99 | pmid=9744974 | doi=10.1056/NEJM199809243391306 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9744974 }} </ref>, the classification of RPGN has changed to involve several types of primary glomerulonephritis that correspond to the quantity and quality of such findings in patients’ sera. ANCA and anti-GBM may co-exist in approximately 20% of the patients.<ref name="pmid7544065">{{cite journal| author=Short AK, Esnault VL, Lockwood CM| title=Anti-neutrophil cytoplasm antibodies and anti-glomerular basement membrane antibodies: two coexisting distinct autoreactivities detectable in patients with rapidly progressive glomerulonephritis. | journal=Am J Kidney Dis | year= 1995 | volume= 26 | issue= 3 | pages= 439-45 | pmid=7544065 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7544065 }} </ref>
====Type I: Anti-GBM Disease====
*Anti-GBM antibody-mediated without pulmonary involvement
*Goodpasture’s disease: Anti-GBM antibody-mediated with pulmonary hemorrhage
====Type II: Immune Complex-Mediated Disease====
====Type III: Pauci-Immune Disease====
ANCA positive
*Idiopathic renal-limited vasculitis / renal-limited necrotizing crescenteric glomerulonephritis (NCGN)
*Granulomatosis with polyangiitis (formerly “Wegener’s granulomatosis")
*Microscopic polyangiitis
*Churg-Strauss syndrome
====Type IV: Mixed Anti-GBM and ANCA Associated Disease====
====Type V: Pauci-Immune====
ANCA negative


==[[Rapidly progressive glomerulonephritis pathophysiology|Pathophysiology]]==
==[[Rapidly progressive glomerulonephritis pathophysiology|Pathophysiology]]==
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==[[Rapidly progressive glomerulonephritis risk factors|Risk Factors]]==
==[[Rapidly progressive glomerulonephritis risk factors|Risk Factors]]==
==[[Rapidly progressive glomerulonephritis screening|Screening]]==


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


==Diagnosis==
==Diagnosis==
 
[[Rapidly progressive glomerulonephritis diagnostic study of choice|Diagnostic study of choice]] | [[Rapidly progressive glomerulonephritis history and symptoms|History and Symptoms]] | [[Rapidly progressive glomerulonephritis physical examination|Physical Examination]] | [[Rapidly progressive glomerulonephritis laboratory findings|Laboratory Findings]] | [[Rapidly progressive glomerulonephritis electrocardiogram|Electrocardiogram]] | [[Rapidly progressive glomerulonephritis x ray|X-Ray Findings]] | [[Rapidly progressive glomerulonephritis echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Rapidly progressive glomerulonephritis CT scan|CT-Scan Findings]] | [[Rapidly progressive glomerulonephritis MRI|MRI Findings]] | [[Rapidly progressive glomerulonephritis other imaging findings|Other Imaging Findings]] | [[Rapidly progressive glomerulonephritis other diagnostic studies|Other Diagnostic Studies]]
[[Rapidly progressive glomerulonephritis diagnostic criteria|Diagnostic Criteria]] | [[Rapidly progressive glomerulonephritis history and symptoms|History and Symptoms]] | [[Rapidly progressive glomerulonephritis physical examination|Physical Examination]] | [[Rapidly progressive glomerulonephritis laboratory findings|Laboratory Findings]] | [[Rapidly progressive glomerulonephritis CT|CT]] | [[Rapidly progressive glomerulonephritis MRI|MRI]] | [[Rapidly progressive glomerulonephritis ultrasound|Ultrasound]] | [[Rapidly progressive glomerulonephritis other imaging findings|Other Imaging Findings]] | [[Rapidly progressive glomerulonephritis other diagnostic studies|Other Diagnostic Studies]]


==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]]
[[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]]


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[[Rapidly progressive glomerulonephritis case study one|Case #1]]
[[Rapidly progressive glomerulonephritis case study one|Case #1]]


==References==
[[Category: (name of the system)]]
<references/>
 
{{Nephrology}}
 
[[Category:Kidney diseases]]
 
[[de:Rasch progressive Glomerulonephritis]]
 
{{WH}}
{{WS}}

Latest revision as of 19:39, 31 July 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2] Jogeet Singh Sekhon, M.D. [3] Syed Ahsan Hussain, M.D.[4]

Synonyms and keywords: Acute cresent glomerulonephritis, RPGN

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Rapidly progressive glomerulonephritis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

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