17 Nephritic Syndrome

Pathogenesis and Symptoms

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• increased hydrostatic pressure in rest of body

• GFR low: The glomeruli are stuffed full of cells, and blood flow slows way down.

• uremia: aka azotemia

Causes

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• membranoproliferative: either nephritic or nephrotic

PSGN

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• deplete complements

• rheumatic fever: master chef with M

• PSGN: post strep glomerulonephritis: chef on phone

• Facial puffs: Facial swelling seen in PSGN

• Cola: Cola colored urine with PSGN

• Calendar: PSGN occurs 2 weeks after Strep infection

• Pencil: Early penicillin for treatment (prevents RF but not PSGN). Even though chef has pencil, he still has symptoms

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• PSGN, lupus, MPGN

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• takes time for immune system

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• hypercellular: inflammation related

• bumpy appearance because in endothelium

• worked their way into sub epithelium

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IgA

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• IgA usually does not activate complement but does here

• weak complement activation, thus no hypocomplementemia

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• IgA stain, like branches of tree

HS Purpura

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• IgA deposition in joints, GI

• IF stain granular

• complement activated via alternative/lectin pathway. IgA does not fix complement

• biopsy shows IgA deposition

DPGN

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• type 1/2: mild; 3-5 serious

• focal: <50% glomerulus involved

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• immune response from anti-dsDNA

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• can be nephrotic, nephritic

RPGN

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• RPGN: common pattern of inflammation in glomeruli representing many diseases

• quickly progress to RF

• elevated BUN:Cr

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RPGN Type I

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• not bumpy

RPGN Type II

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RPGN type III

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• Pauci-immune: no immune staining

Alport

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• male child with triad

• Electron microscopy of a renal biopsy shows irregular thinning of the glomerular basement membrane (GBM) with splitting/lamination of the lamina densa, giving the GBM a unique “basketweave” appearance.