nephritic syndrome

Overview of 
Nephritic Syndrome

Characterized by inflammation in the glomeruli.

# Hematuria
# Azotemia : exessive nitrogen compounds in the blood as urea and creatinine. 
Azote = nitrogen , hemia = blood.
# Oliguria.
# Hypertension.
# mild proteinurea and edema.

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#1 Acute proliferative poststreptococcal glomerulonephritis:
PSGN

Pathogenesis: immune comlex-mediated disorder characterized by deposition of Ab-Ag comlexes containing proteins derived from some bacteria. These comlexes lead to activiation of complement system and injury to GBM.

Morpholgy: #Hypercellularity: due to neutrophil and monocytes infiltration. ( Acute inflammation)

# Cell proliferation, mesangial ,epithelial and endothelial.

#Granular mesangial and GBM IgG and C3.
# Subendothelial humplike deposits. زي السنام

Acute proliferative glomerulonephritis. A, Normal glomerulus. B, Glomerular hypercellularity is due to intracapillary leukocytes and proliferation of intrinsic glomerular cells. C, Typical electron-dense subepithelial “hump” and a neutrophil in the lumen. D, Immunofluorescent stain demonstrates discrete, coarsly granular deposits of complement protein C3, corresponding to “humps” illustrated in part C.

Clinical course: # occurs after pharyngeal or cutaneous streptococcal infection.

# Group A, B-hemolytic streptococci are nephritogenic.

# increased anti-streptococcal Ab titre. Decreased C3 concentrations.

# 95% ---> recover

# 1% ---> RPGN

# 2% ---> chronic renal failure

#2 Rapidly progrissive ( crescentic ) glomerulonephritis :

RPGN

TYPE I (ANTI-GBM ANTIBODY)

Renal limited    Goodpastursyndrome

TYPE II (IMMUNE COMPLEX)

Idiopathic    Post-infectious glomerulonephritis    Lupus nephritis    Henoch-Schönlein purpura (IgA nephropathy)    Others

TYPE III (PAUCI-IMMUNE)

ANCA-associated    Idiopathic    Wegener granulomatosis    Microscopic polyangiitis

Pathogenesis: rapid and progressive reanl decline.

Type I RPGN: Anti-GBM antibody 
#linear IgG and C3 GBM deposits.
#can react with pulmonary alveolar BM causing hemorrage ( goodpasture syndrome )

Type II RPGN: immune-complex mediated disease
#a complication of PSGN, LUPUS or ideopathic.
#crescent formation from proliferation of parietal cells.

Type III RPGN: ANCA-associted
#No anti-GBM antibody or immune complexes.
#instead, there is anti neutrophil cytoplasmic antibody.

Crescentic glomerulonephritis (PAS stain). Note the collapsed glomerular tufts and the crescent-shaped mass of proliferating parietal epithelial cells and leukocytes internal to Bowman capsule.

Morphology (( Little Moon )) :)