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| {{Infobox_Disease
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| | Name = {{PAGENAME}}
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| | DiseasesDB = 3165
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| | ICD10 = N00-N08 with .7 suffix
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| | eMedicine_mult = {{eMedicine2|med|890}} |
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| {{Rapidly progressive glomerulonephritis}} | | {{Rapidly progressive glomerulonephritis}} |
| | '''For patient information, click [[Rapidly progressive glomerulonephritis (patient information)|here]]''' |
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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
| | {{CMG}}; {{AE}} {{AEL}} {{JSS}} {{SAH}} |
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| {{CMG}} | | {{SK}} Acute cresent glomerulonephritis, RPGN |
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| ==[[Rapidly progressive glomerulonephritis overview|Overview]]== | | ==[[Rapidly progressive glomerulonephritis overview|Overview]]== |
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| | ==[[Rapidly progressive glomerulonephritis historical perspective|Historical Perspective]]== |
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| ==[[Rapidly progressive glomerulonephritis classification|Classification]]== | | ==[[Rapidly progressive glomerulonephritis classification|Classification]]== |
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| ==[[Rapidly progressive glomerulonephritis differential diagnosis|Differentiating Rapidly progressive glomerulonephritis from other Diseases]]== | | ==[[Rapidly progressive glomerulonephritis differential diagnosis|Differentiating Rapidly progressive glomerulonephritis from other Diseases]]== |
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| | ==[[Rapidly progressive glomerulonephritis epidemiology and demographics|Epidemiology and Demographics]]== |
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| ==[[Rapidly progressive glomerulonephritis risk factors|Risk Factors]]== | | ==[[Rapidly progressive glomerulonephritis risk factors|Risk Factors]]== |
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| | ==[[Rapidly progressive glomerulonephritis screening|Screening]]== |
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| ==[[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]]== |
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| ==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]] | |
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| ==Treatment== | | ==Treatment== |
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| [[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]] |
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| '''Rapidly progressive glomerulonephritis''' ('''RPGN''') is a [[syndrome]] of the [[kidney]] that, if left untreated, rapidly progresses into [[acute renal failure]] and death within months. In 50% of cases, RPGN is associated with an underlying disease such as [[Goodpasture syndrome]], [[systemic lupus erythematosus]], or [[Wegener granulomatosis]]; the remaining cases are [[idiopathic]]. Regardless of the underlying cause, RPGN involves severe injury to the kidney's [[glomeruli]], with greater than 50% of the glomeruli containing characteristic crescent-shaped [[scar]]s. Because of this microscopic feature, RPGN is also called '''crescentic glomerulonephritis'''.
| | [[Category: (name of the system)]] |
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| Patients with RPGN have blood in the urine ([[hematuria]]), urinary protein ([[proteinuria]]), and occasionally high blood pressure ([[hypertension]]) and [[edema]]. The clinical picture is consistent with [[nephritic syndrome]], although the degree of proteinuria may occasionally exceed 3 [[gram|g]]/24 h, a range associated with [[nephrotic syndrome]]. Untreated disease may progress to decreased urinary volume ([[oliguria]]), which is associated with poor kidney function.
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| RPGN is classified into three types, all of which involve immune-mediated damage to the glomeruli. In type I RPGN, which accounts for approximately 20% of RPGN cases, injury is caused by [[antibodies]] directed against the [[glomerular basement membrane]]. Type II RPGN accounts for roughly 25% of RPGN cases and is characterized by the deposition of [[immune complex]]es in the glomerulus. The remainder of RPGN cases are type III, or pauci-immune RPGN, which features antibodies directed against [[neutrophil]]s ([[anti-neutrophil cytoplasmic antibodies]], ANCA).
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| Treatment depends on the underlying disease process. For example, [[plasmapheresis]], [[corticosteroid]]s, and [[cytotoxic]] drugs may promote recovery in Goodpasture syndrome, a cause of type I RPGN. Despite even early treatment, however, many patients with RPGN may ultimately require [[dialysis]] and possibly [[renal transplant]].
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| ==Classification==
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| ===Type I===
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| 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>
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| 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"/>
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| ===Type II===
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| 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"/>
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| ===Type III===
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| 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"/>
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| 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"/>
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| ==Signs and symptoms==
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| Most types of RPGN are characterized by severe and rapid loss of kidney function featuring severe [[hematuria]] (blood in the urine), [[red blood cell cast]]s in the urine, and [[proteinuria]] (protein in the urine), sometimes exceeding 3 g protein/24 h, a range associated with [[nephrotic syndrome]]. Some patients also experience [[hypertension]] (high blood pressure) and [[edema]]. Severe disease is characterized by pronounced [[oliguria]] or [[anuria]], which portends a poor prognosis.<ref name="robbins"/>
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| When the cause of RPGN is Goodpasture syndrome or vasculitis that involves the lungs (such as Wegener granulomatosis), the lungs and upper airway may be involved. Patients with such underlying diseases may present with [[cough]], [[hemoptysis]], [[dyspnea]], nasal bleeding, obstruction, or [[sinusitis]].{{Fact|date=October 2007}}
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| [[Serology|Serum analysis]] often aids in the diagnosis of a specific underlying disease. The presence of anti-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 ANCA-positive serum.<ref name="robbins"/>
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| ==Crescent formation==
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| Despite the wide variety of diseases that cause RPGN, all types of RPGN are characterized by glomeruluar injury and the formation of crescents. Severe injury and GBM rupture leads to the leakage of [[plasma protein]]s through the GBM. Of these proteins, [[fibrin]] is thought to contribute most strongly to crescent formation. [[Epithelial cell]]s lining the [[Bowman capsule]] respond to the leaked fibrin and proliferate. Infiltrating [[white blood cell]]s such as [[monocyte]]s and [[macrophage]]s may also proliferate. These proliferating cells surround and compress the glomerulus, forming a crescent-shaped scar that is readily visible on [[light microscopy]] of a [[renal biopsy]].<ref name="robbins"/>
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| ==References==
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| <references/>
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| {{Nephrology}}
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| [[Category:Kidney diseases]]
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| [[de:Rasch progressive Glomerulonephritis]]
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| {{jb1}}
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| {{WH}}
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| {{WS}}
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