Rapidly progressive glomerulonephritis

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Rapidly progressive glomerulonephritis
Other namesCrescentic glomerulonephritis[1]
Histopathological image of crescentic glomerulonephritis in a patient with MPO-ANCA positive rapid progressive glomerulonephritis. Hematoxylin & eosin stain.
SpecialtyNephrology
SymptomsHematuria[2]
TypesType I, II and III[3]
Diagnostic methodSerum analysis[2]
TreatmentCorticosteroids

Rapidly progressive glomerulonephritis (RPGN) is a

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).[7]

Signs and symptoms

Most types of RPGN are characterized by severe and rapid loss of kidney function with marked

red blood cell casts in the urine; and proteinuria sometimes exceeding three grams in twenty-four hours, a range associated with nephrotic syndrome. Some patients also experience hypertension and edema. Severe disease is characterized by pronounced oliguria or anuria, which portends a poor prognosis.[2]

Pathophysiology

It is thought that

podocytes.[9]

Diagnosis

anti-neutrophil cytoplasmic antibodies (ANCA)-positive serum.[2]

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.[10]

Classification

Crescentic glomerulonephritis

RPGN can be classified into three types, based upon the immunofluorescence patterns:[3]

Type I

Accounting for approximately 3

autoantibodies directed against type IV collagen (specifically, the noncollagenous region of its α3 chain)[2] 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.[2]

Type II

Characterized by deposition of

systemic lupus erythematosus, acute proliferative glomerulonephritis, Henoch–Schönlein purpura, and IgA nephropathy—that involves the glomerulus may progress to RPGN if severe enough.[2]

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.[2]

Treatment

Therapy consists of a combination of rituximab,

corticosteroids, and cyclophosphamide, with a substitution of azathioprine for cyclophosphamide after a ninety-day initial period being another option.[13] When remission is achieved, immunosuppressants are still used,[14] usually corticosteroids with azathioprine or rituximab infusions.[14]

Epidemiology

The

incidence rate of rapidly progressive glomerulonephritis is approximately 3.9 individuals per million.[15]

References

  1. ^ RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Rapidly progressive glomerulonephritis". www.orpha.net. Retrieved 31 July 2019.{{cite web}}: CS1 maint: numeric names: authors list (link)
  2. ^
    ISBN 978-0-7216-0187-8
    .
  3. ^
    ISBN 978-1-4200-8478-8
    .
  4. ^ a b TheFreeDictionary > rapidly progressive glomerulonephritis Citing: McGraw-Hill Concise Dictionary of Modern Medicine. 2002
  5. ^ a b c eMedicine > Glomerulonephritis, Crescentic Author: Malvinder S Parmar. Updated: Sep 25, 2008
  6. ^ "rapidly progressive glomerulonephritis" at Dorland's Medical Dictionary
  7. PMID 32132388
    .
  8. ^ "Rapidly Progressive Glomerulonephritis: Background, Pathophysiology, Epidemiology". 2018-04-05.
  9. PMID 4125698
    .
  10. PMID 21751663
    . Retrieved 31 October 2015.
  11. ^ https://www.msdmanuals.com/professional/multimedia/table/classification-of-rapidly-progressive-glomerulonephritis-based-on-immunofluorescence-microscopy
  12. ^ https://www.msdmanuals.com/professional/multimedia/table/classification-of-rapidly-progressive-glomerulonephritis-based-on-immunofluorescence-microscopy
  13. ^
    PMID 25815169
    .
  14. ^
    PMID 28842398
    .
  15. PMID 11007827
    .

Further reading

External links

Scholia has a topic profile for Rapidly progressive glomerulonephritis.
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