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NEPHRITIC SYNDROME - Coggle Diagram
NEPHRITIC SYNDROME
Glomerular
inflammation
& bleeding
Biopsy reveals hypercellular, inflamed glomeruli
Immune-complex deposition activates complement; C5a attracts neutrophils, which mediate damage.
Limited proteinuria (< 3.5 g/day)
Oliguria and azotemia
Salt retention with periorbital edema and hypertension
RBC casts and dysmorphic RBCs in urine
RAPIDLY PROGRESSIVE
GLOMERULONEPHRITIS
Anti-GBM auto-antibodies (
IgG
)
vs
alpha-3 chain
of
type IV collagen
+
deposits of C3
+ leukocyte
infiltration → Crescentic glomerulonephritis
HY!!
Hematuria + hemoptisis
-
Adult Young males
Goodpasture Sx: Autoantibodies-Cross reaction with alveolar basement membrane (also type IV collagen)
Type II hypersensitivity
with poor prognosis
Crescent formation
in Bowman space on H&E
/
Linear formation
in Immunofluorescence
*Crescents consist of fibrin and plasma proteins (C3b) with glomerular parietal cells, monocytes, macrophages
IgA NEPHROPATHY
(BERGER DISEASE)
HY: IgA immune
complex deposition in
mesangium of glomeruli
IgA immune complex
seen on Immunofluoresence
Features:
Presents in childhood: gross hematuria with RBC casts after mucosal infections (gastroenteritis)
May slowly progress to renal failure
Most common
nephropathy worldwide
POSTSTREPTOCOCCAL
GLOMERULONEPHRITIS (PSGN)
Hypercellular inflamed
glomeruli on H&E
HY: Mediated by immune
complex deposition IgG, IgM C3
Arises after group A β- hemolytic
streptococcal infection of
the skin (impetigo) or pharynx
2-3 weeks after infection
Hematuria + oliguria + hypertension + and periorbital edema
Usually children
, but adults too
Supportive care
Children 1% progress to renal failure
Adults 25% develop rapidly progressive glomerulonephritis (RPGN)
Nephritogenic strains
-
Nephritogenic antigens
Granular deposits Immunofluorescence
& subendothelial HUMPS on EM, HY
Complement activation
via alternate pathway
ALPORT SYNDROME
Inherited defect in type IV collagen
HY: Irregular thinning /thickening and splitting of the glomerular basement membrane Basket-weave appearance
Initially no deposits, but later irregular deposits of IgG, IgM and/or C3
Clinical: isolated hematuria, sensory hearing loss, and ocular disturbances
Most commonly X-linked
DIFUSSE PROLIFERATIVE
GLOMERULONEPHRITIS
DPGN and MPGN often present
as nephritic and nephrotic
syndromes concurrently
Subendothelial, subepithelial or intramembranous
IgG-based ICs often with C3 deposition
“Wire looping” of capillaries
GRANULAR PATTERN IN IMMUNOFLUORESENCE
HY: glomerular deposition of DNA-anti-DNA immune complexes Most common and severe type in SLE(most common cause of death in patients with SLE)