ACUTE NEPHRITIC NEPHROTIC SYNDROME

SYNDROME
Clinically, presented as: Clinically: Causes: Primary glomerular disease: Secondary systemic cause:
# Hematuria # Heavy proteinuria capillary permeability # some are directly According to morphology, -diabetes mellitus
# Proteinuria # Hypoproteinemia 2ry to loss of protein targeting the -minimal change disease -amyloidosis
# Oliguria & azotemia # Generalized edema plasma osmotic glomeruli(1ry glomerular -focal segmented -SLE
# Hypertension pressurecompensatory secretion of disease) glomerularsclerosis -infections(HBV, malaria,
# Mild edema aldosteronesalt and water retention # some are due to -membronaous schistosomiasis)
# Hyperlipidemia synthesis of lipoprotein systemic disease that glomerulonephritis -drugs, maignancies,
# Lipiduria GBM permeability to affect glomeruli & other -membranoproliferative hereditary
lipoprotein. tissue(2ry) glomerulonephritis
-Ig A nephropathy

POST STREPTOCOCCAL MINIMAL CHANGES FOCAL SEGMENTED MEMBRANOUS MEMBRANOPROLIFERATIVE IG A NEPHROPATHY

(ACUTE DIFFUSE GLOMERULOSCLEROSIS GLOMERULONEPHRITIS GLOMERULONEPHRITIS
PROLIFERATIVE GN)
INCIDENCE
Common disorder Main cause of NS NS in 15% of adults and Most frequent cause of 5-10% of cases of primary NS Most common glomerular
among children that among children children NS among adults in in children and adults disease worldwide
follow infection of skin < 15 years western countries Peak: children and young
or URT by nephritogenic adults
strain of B-haem.
streptococci.

CHARACTERISTICS HISTOLOGICAL FEATURES

Normal looking Focal & segmental Accumulation of immune Glomerular hypercellularity Deposition of immune
glomeruli with LM & obliteration of capillary complexes in with thickening of GBM. complex formed
diffuse loss of loop by deposition of subepithelial zone of predominantly of Ig A
epithelial foot collagen(sclerosis)+accu glomerular capillaries.
processes by EM. mulation of lipid &
proteinaceous material
 PATHOGENESIS
-immune complex -might be 1ry -injury to epithelial cell Immune complex -Type I caused by circulating -genetic or aquired
deposited within the epithelial injury(no lead to focal mediated disease: immune complexes abnormality of immune
glomeruli initiate immune complex hyperpermeable foci -1ry: due to in situ -Type II: autoimmune disease. regulation leading to
inflammation by deposition) entrapment of plasma deposition of immune The patients serum has factor mucosal Ig A synthesis in
activation of -disorder in T- protein & lipid complexes against renal called C3 nephritic factor that response to resp of GIT
complement system lymphocytes leading -result in mesangial cell autoantigen activate alternate exposure to environmental
to elaboration of reaction with mesangial -2ry: due to circulating complement pathway with agents. Ig A and Ig A
cytokines that affect matrix immune complexes elaboration of biologically complex then get trapped
synthesis of against exogenous active complement pathway. within mesangium,
nephrin. antigen activate alternate
Consequently, there complement pathway &
is loss of podocyte. initiate glomerular injury.
CLINICAL PICTURES
1.nephritic syndrome In NS, there is 1.non selective NS, sometimes non- Both types present mainly by 1.gross hematuria that
2.low serum neither proteinuria nephrotic range non- NS, sometimes with non- occur within 1-2 days of
complement level hypertension nor 2.may progress to NS selective proteinuria nephrotic range proteinuria nonspecific URT/GIT
3.high titre of anti- hematuria 3.may develop infection. Hematuria lasts
streptolysin O(ASO) in hypertension & several days then subsides.
serum hematuria Its recurs every few
months & associated with
loin pain.
2.less 10% with NS
PROGNOSIS AND FATE
Depend on pts age 7 Good prognosis Poor response to Proteinuria usually does Both types have poor Has remitting & relapsing
causative agents with excellent corticosteroids with not respond to prognosis, more than 50% of course. Rarely resolves but
-good prognosis with response to progression to renal corticosteroids. However them progress to chronic Gn many patients maintain
streptococcal infection & corticosteroid failures within 10 years in it has an indolent course after 10 years. normal renal functions for
among children. therapy in > 90% of about 50% of cases. & only about 40% of pts Type II has tendency to recur decades. 50% slowly
-children: usually recover affected children & Lesion tend to recur in progress to renal failure in renal allograft. progress to chronic renal
(90%) after sveral weeks less figures in adult. renal allograft. failure within 20 years.
- few cases developed
rapidly progressive Gn or
chronic renal disease.
-in adult: 15-50%
develop chronic Gn
within few years
 MICROSCOPIC PICTURES
LIGHT MICROSCOPE
-Diffuse in mesangial Normal looking Some show segmental Diffuse thickening of Both types have similar LM. Not specific. The glomeruli
cells with infiltration by glomeruli obliteration of capillary GBM, normal glomerular Glomeruli are diffusely commonly show mesangial
neutrophils leading to loop with mesangial cellularity. enlarged, proliferation
compression of capillary matrix, collapsed GBM & hypercellular(mesangial
lumina (bloodless accumulation of lipid & proliferation) & leukocytes
glomeruli) proteinaceous material. with diffuse thickening of
-cresent GBM that displayed double
formation(proliferation contour(tram-track
of parietal cells) appearance) by silver stain.
FLUOROSCENCE MICROSCOPE
Granular deposits of Ig G No deposits Usually negative Typical granular deposits Type I: granular deposits of Ig Intense mesangial staining
and C3 along capillary of Ig & complement & complement along capillary for Ig A
wall along GBM wall & mesangium
Type II: granular deposits of
C3 allong capillary with
absence of Ig deposition.
ELECTRON MICROSCOPE
Scattered subepithelial Diffuse loss of Focal segmental in Subepithelial deposits Type I: marked mesangial Electron dense deposits
deposits shaped like epithelial foot mesangial matrix with nestle against GBM & hypercellularity + mesangial within the mesangium.
humps processes prominent injury of separated from each interposition between
overlying podocyte other by small spike-like capillary wall splllitting
protrusion of GBM associat with subendothelial &
matrix mesangial electron dense
deposits
Type II: ribbon-like dense
deposits in the centre of
thickened GBM.