THE KIDNEY

GLOMERULAR DISEASES

Gopal

Dr.Venu
22/10/08

In primary glomerulonephritis,
the kidney is the principal organ
involved
In secondary Glomerular
diseases, the kidney is one of
many organ systems damaged by
a systemic disease.
The chronic glomerulonephritis is
the most common cause of
chronic renal failure.

Acute diffuse proliferative

glomerulonephritis

Post-streptococcal
Non- Post streptococcal.

Rapidly progressive [ crescentric]

glomerulonephritis.
Membranous glomerulopathy.
Minimal change disease.
Focal segmental glomerulosclerosis
Membrano - Proliferative glomerulonephritis
Ig A nephropathy.
Chronic glomerulonephritis.

SYSTEMIC DISEASE WITH

GLOMERULAR INVOLVEMENT

Systemic lupus erythematosus.

Diabetes mellitus
Amyloidosis.
Good pasture syndrome.
Microscopic polyarteritis/Polyangitis.
Wegeners granulomatosis.
Henoch-scholein purpura.
Bacterial endocarditis.

HEREDITARY DISORDERS

Alport syndrome.
Thin basement membrane disease.
Fabry disease.

Pathogenesis of Glomerular Injury:

The immune mechanism predominate in

Glomerular Injury.

IMMUNE
MECHANISM
The glomerular deposition of

antigen antibody complexes is a

major mechanism of glomerular
injury.
The antibodies can be directed against
injury.
Fixed intrinsic antigens .

Anti- glomerular basement membrane

nephritis
autoimmune disease in which
antibodies are towards non- collagenous
domain of the alpha3 chain of GBM type IV

This yields a linear immunofluroscence

staining pattern.
HEYMANNS NEPHRITIS OF RATS:
The antibodies react with a large 330KD
megalin protein antigen expressed on
visceral epithelial cells
The resultant lesion exhibit sub epithelial
deposits of antigen antibody complexes
resembling those in human
membraneous glomerulonephritis.
planted circulating exogenous
[e.g:- infectious agent ] or endogenous

[ e.g:- DNA] antigens can occur.

CIRCULATING IMMUNE
COMPLEXES
Antigens may be endogenous

[e.g.:[e.g:-

thyroglobin] or exogenous
infectious agent]
Complexes are usually deposited
subendothelially/ in the mesangium and
yield a granular immunofluroscence
pattern.
Cytotoxic antibodies.
Cell-mediated immune injury.
Activation of alternative complement
pathway.

Acute Proliferative [Post Streptococcal,

Post-Infectious] Glomerulonephritis :
Characterized by acute nephritic
syndrome
1-4 weeks after streptococcal pharyngitis
infection.
Antibody mediated disease.
Types 12,4,1 strains of group A BHemolytic streptococci are
nephrotogenic .

Biopsy specimen show diffuse

Glomerulonephritis and global hyper
cellularity due to endothelial
proliferation, mesangial and epithelial
cell infiltration.
Immunofluroscence show granular I gG,
IgM and C3 deposition and electron
microscopy show subepithelial hump
like structures.
The serum anti-streptococcal antibody
levels are elevated and decrease of C3
serum complements level.

Characterised by cellular accumulation in

bowman space in the form of crescents
accompanied by a rapidly, Progressive decline
in renal function.

The RPGN is divided into 3 broad groups.

Type I RPGN is an anti-GBM disease is

Characterised by linear IgG deposits in the GBM.

In some,anti-GBM antibodies react pulmonary

alveolar basement membraneous to produce
pulmonary haemorrhages

The good pasture antigen is a peptide with in

the noncollagenous domain of the 3
chain of type IV collagen.
Type II RPGN is an immune-complex mediated
disease: The immunofluroscence shows
characterstic [lumpy bumpy] granular
staining.
Type III RPGN:- also called Pauci -immune
Antineutrophilic cytoplasmic
antibodies(ANCA) associated.
Wegener granulomatosis.
Microscopic polyarteritis nodosa.

MORPHOLOG
Y

There is a distinctive crescents

formed by parietal cell proliferation
and monocyte and macrophage
migration into bowmans space.
The electron microscopy discloses
subepithelial deposits with rupture in
GBM

Haematuria, moderate proteinuria and

variable hypertension and oedema.
Good pasture syndrome show recurrent
haemoptysis.
Serum analyses for anti-GBM
antibodies and ANCA are helpful in
diagnosis.

It is the major cause of nephrotic syndrome in

adults.
It show diffuse glomerular capillary wall
thickening due to deposition of
immunoglobulin containing electron dense
material along the subepithelial side of G.B.M
The disease is idiopathic in 85% of patients.
The remaining 15% of membraneous G.N is
associated with underlying malignant tumors
SLE exposure to gold ,mercury, drug
infections.

MORPHOLOG
Y

There is diffuse thickening of the

capillary wall, hence the term
membraneous .
Immunofluroscence there is diffuse
granular staining pattern, there are
subepithelial GBM deposits, which
gets incorporated into GBM and
assume intramembraneous location.

CLINICAL
FEATURES:

This usually starts with the insidious

onset of nephrotic syndrome. 40% of
cases progress to renal insufficiency
over an unpredictable time span of 2 to
20 years.