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- Acute glomerulonephritis (GN) is an inflammatory process involving the glomerulus of the kidney, resulting in a clinical syndrome consisting of hematuria, proteinuria, hypertension, and renal insufficiency.
- Acute GN may be 1 of many primary diseases, or it may present as part of a systemic disease:
- Postinfectious GN
- IgA nephropathy–Henoch Schönlein purpura
- Antiglomerular basement membrane disease (anti-GBM disease)
- Antineutrophil cytoplasmic antibody (ANCA)-associated GN
- Membranoproliferative GN (MPGN)
- Lupus nephritis
- Cryoglobulin-associated GN
- Clinical severity ranges from asymptomatic microscopic or gross hematuria to a rapid loss of kidney function (rapidly progressive GN [RPGN]).
Urgent investigation and treatment are required to avoid irreversible loss of kidney function.
- Postinfectious GN:
- Most commonly follows group A β-hemolytic Streptococcus infection, but can occur as a result of other infections
- Onset occurs 1–3 weeks after an infectious process (throat or skin).
- Accounts for 80% of acute GN in children
- IgA nephropathy:
- Most common form of primary acute GN
- Occurs mainly in the 2nd and 3rd decades
- Male > Female (3:1)
- Incidence differs geographically: Asia > US
- Anti-GBM disease:
- Also known as Goodpasture disease
- A noted cause of the pulmonary–renal syndrome
- Occurs most commonly in the 2d or 3rd decade
- Male > Female (6:1)
- ANCA-associated GN:
- Uncommon: Often has a relapsing and remitting course
- 3 disease presentations:
- Wegener granulomatosis
- Churg-Strauss disease
- Microscopic polyangiitis
- Older patients are more commonly affected, although this GN can affect any age group.
- May be primary or secondary
- May present in the setting of a systemic viral or rheumatic illness
- Lupus nephritis:
- 30–70% of systemic lupus patients will have renal involvement.
- Cryoglobulin-associated vasculitis:
- 80% of cases are associated with hepatitis C infection.
- Epidemics of nephritogenic strains of streptococci are triggers for postinfectious GN.
- Hepatic cirrhosis and celiac disease place patients at risk for IgA nephropathy.
- Anti-GBM disease has been associated with influenza A infection and inhaled hydrocarbon solvent exposure.
- ANCA-associated GN is increased in settings where there is increased silica exposure (i.e., earthquakes and farming).
- Infection with hepatitis B and/or C are known to be associated with MPGN.
- Infection with hepatitis C is a risk factor for developing cryoglobulinemic GN.
- Mutations in alternate complement pathway genes are associated with MPGN.
Genetic factors are likely to play a role in susceptibility to many of the acute GNs, although these have not been sufficiently defined to be useful clinically.
Early detection is paramount.
- In general, an immunologic mechanism triggers inflammation and proliferation of glomerular tissue.
- Postinfectious GN: Host immune reaction to nephritogenic strains of streptococci is trigger.
- IgA nephropathy: Relates to an abnormal glycosylation of IgA
- Anti-GBM disease: Caused by autoantibodies that target type IV collagen of basement membranes
- ANCA-associated GN: Autoantibodies against neutrophil granules are involved in the pathogenesis.
- MPGN: An immune or genetic etiology is presumed, which triggers renal deposits and inflammation.
- Lupus nephritis: An immune complex-mediated glomerular disease
- Cryoglobulin-associated GN: An immune etiology is presumed, but not clearly defined.