ACUTE NEPHRITIC

SYNDROME
Clinically, presented as:
# Hematuria
# Proteinuria
# Oliguria & azotemia
# Hypertension
# Mild edema

NEPHROTIC SYNDROME
Clinically:
# Heavy proteinuria
capillary permeability
# Hypoproteinemia 2ry to loss of protein
# Generalized edema
plasma osmotic
pressurecompensatory secretion of
aldosteronesalt and water retention
# Hyperlipidemia
synthesis of lipoprotein
# Lipiduria
GBM permeability to
lipoprotein.

Causes:
# some are directly
targeting the
glomeruli(1ry glomerular
disease)
# some are due to
systemic disease that
affect glomeruli & other
tissue(2ry)

Primary glomerular disease:

According to morphology,
-minimal change disease
-focal segmented
glomerularsclerosis
-membronaous
glomerulonephritis
-membranoproliferative
glomerulonephritis
-Ig A nephropathy

Secondary systemic cause:

-diabetes mellitus
-amyloidosis
-SLE
-infections(HBV, malaria,
schistosomiasis)
-drugs, maignancies,
hereditary

POST STREPTOCOCCAL
(ACUTE DIFFUSE
PROLIFERATIVE GN)

MINIMAL CHANGES

FOCAL SEGMENTED
GLOMERULOSCLEROSIS

MEMBRANOUS
GLOMERULONEPHRITIS

MEMBRANOPROLIFERATIVE
GLOMERULONEPHRITIS

IG A NEPHROPATHY

Common disorder
among children that
follow infection of skin
or URT by nephritogenic
strain of B-haem.
streptococci.

Main cause of NS
among children
< 15 years

NS in 15% of adults and

children

INCIDENCE
Most frequent cause of
NS among adults in
western countries

5-10% of cases of primary NS

in children and adults

Most common glomerular

disease worldwide
Peak: children and young
adults

Normal looking
glomeruli with LM &
diffuse loss of
epithelial foot
processes by EM.

CHARACTERISTICS HISTOLOGICAL FEATURES

Focal & segmental
Accumulation of immune Glomerular hypercellularity
obliteration of capillary
complexes in
with thickening of GBM.
loop by deposition of
subepithelial zone of
collagen(sclerosis)+accu
glomerular capillaries.
mulation of lipid &
proteinaceous material

Deposition of immune
complex formed
predominantly of Ig A

-immune complex
deposited within the
glomeruli initiate
inflammation by
activation of
complement system

-might be 1ry
epithelial injury(no
immune complex
deposition)
-disorder in Tlymphocytes leading
to elaboration of
cytokines that affect
synthesis of
nephrin.
Consequently, there
is loss of podocyte.

1.nephritic syndrome
2.low serum
complement level
3.high titre of antistreptolysin O(ASO) in
serum

In NS, there is
neither
hypertension nor
hematuria

Depend on pts age 7

causative agents
-good prognosis with
streptococcal infection &
among children.
-children: usually recover
(90%) after sveral weeks
- few cases developed
rapidly progressive Gn or
chronic renal disease.
-in adult: 15-50%
develop chronic Gn
within few years

Good prognosis
with excellent
response to
corticosteroid
therapy in > 90% of
affected children &
less figures in adult.

PATHOGENESIS
-injury to epithelial cell
Immune complex
lead to focal
mediated disease:
hyperpermeable foci
-1ry: due to in situ
entrapment of plasma
deposition of immune
protein & lipid
complexes against renal
-result in mesangial cell
autoantigen
reaction with mesangial -2ry: due to circulating
matrix
immune complexes
against exogenous
antigen

1.non selective
proteinuria
2.may progress to NS
3.may develop
hypertension &
hematuria

CLINICAL PICTURES
NS, sometimes nonnephrotic range nonselective proteinuria

PROGNOSIS AND FATE

Poor response to
Proteinuria usually does
corticosteroids with
not respond to
progression to renal
corticosteroids. However
failures within 10 years in it has an indolent course
about 50% of cases.
& only about 40% of pts
Lesion tend to recur in
progress to renal failure
renal allograft.

-Type I caused by circulating

immune complexes
-Type II: autoimmune disease.
The patients serum has factor
called C3 nephritic factor that
activate alternate
complement pathway with
elaboration of biologically
active complement pathway.

-genetic or aquired
abnormality of immune
regulation leading to
mucosal Ig A synthesis in
response to resp of GIT
exposure to environmental
agents. Ig A and Ig A
complex then get trapped
within mesangium,
activate alternate
complement pathway &
initiate glomerular injury.

Both types present mainly by

NS, sometimes with nonnephrotic range proteinuria

1.gross hematuria that

occur within 1-2 days of
nonspecific URT/GIT
infection. Hematuria lasts
several days then subsides.
Its recurs every few
months & associated with
loin pain.
2.less 10% with NS

Both types have poor

prognosis, more than 50% of
them progress to chronic Gn
after 10 years.
Type II has tendency to recur
in renal allograft.

Has remitting & relapsing

course. Rarely resolves but
many patients maintain
normal renal functions for
decades. 50% slowly
progress to chronic renal
failure within 20 years.

-Diffuse in mesangial
cells with infiltration by
neutrophils leading to
compression of capillary
lumina (bloodless
glomeruli)
-cresent
formation(proliferation
of parietal cells)

Normal looking
glomeruli

Granular deposits of Ig G
and C3 along capillary
wall

No deposits

Scattered subepithelial
deposits shaped like
humps

Diffuse loss of
epithelial foot
processes

MICROSCOPIC PICTURES
LIGHT MICROSCOPE
Some show segmental
Diffuse thickening of
obliteration of capillary
GBM, normal glomerular
loop with mesangial
cellularity.
matrix, collapsed GBM &
accumulation of lipid &
proteinaceous material.

Usually negative

FLUOROSCENCE MICROSCOPE
Typical granular deposits
of Ig & complement
along GBM

ELECTRON MICROSCOPE
Focal segmental in
Subepithelial deposits
mesangial matrix with
nestle against GBM &
prominent injury of
separated from each
overlying podocyte
other by small spike-like
protrusion of GBM
matrix

Both types have similar LM.

Glomeruli are diffusely
enlarged,
hypercellular(mesangial
proliferation) & leukocytes
with diffuse thickening of
GBM that displayed double
contour(tram-track
appearance) by silver stain.

Not specific. The glomeruli

commonly show mesangial
proliferation

Type I: granular deposits of Ig

& complement along capillary
wall & mesangium
Type II: granular deposits of
C3 allong capillary with
absence of Ig deposition.

Intense mesangial staining

for Ig A

Type I: marked mesangial

hypercellularity + mesangial
interposition between
capillary wall splllitting
associat with subendothelial &
mesangial electron dense
deposits
Type II: ribbon-like dense
deposits in the centre of
thickened GBM.

Electron dense deposits

within the mesangium.