ACUTE PROLIFERATIVE GLOMERULONEPHRITIS AND RAPIDLY PROGRESSIVE GN

YASH SARAN
ROLL NO. 115
 NEPHRITIC SYNDROME
Nephritic syndrome is the typical clinical presentation of most
proliferative types of GN.
The main clinical features of nephritic syndrome include :

• HEMATURIA (SMOKY OR COLA COLOURED URINE)

• PROTEINURIA( Subnephrotic range)
• AZOTEMIA
• HYPERTENSION
• OLIGURIA
• OEDEMA
 ACUTE PROLIFERATIVE GN
(POST-INFECTIOUS/POST-STREPTOCOCCAL GN)

• Characterized histologically by diffuse

proliferation of glomerular cells, and
infiltration by leukocytes.
• Immune complex mediated disease and the
inciting antigen is of two types:
Exogenous Endogenous
POST-INFECTIOUS GN NEPHRITIS OF SLE
 PATHOGENESIS
• It follows streptococcal infection rather than direct
primary infection of the kidney.
• Primary streptococcal infection usually involves the
pharynx or the skin.
• Only certain strains of group A β-hemolytic streptococci
are nephritogenic. Most common are types 12, 4 and 1.
• Main streptococcal antigenic component responsible for
immune reaction is streptococcal pyogenic exotoxin B
(SpeB)
• This leads to formation of immune complexes at the
glomerular basement membrane leading to
inflammatory response.
Evidences to support Immunological basis
• Latent period: It ranges from 1-4 weeks, between
the streptococcal infection and beginning of GN and
is compatible with the time required for the
production of antibodies and the immune complex
formation.
• Antibodies against streptococcal antigens : Found to
be increased in majority of patients.
• Hypocomplementemia : Observed in more than 90%
of patients due to activation and utilization of
immune complexes.
• Immune complex deposits
• Streptococcal antigens in the glomeruli
 Mechanism Of Damage
• Immune complexes are formed in the circulation
and gets deposited within glomeruli.
• These immune complexes initiate inflammation.
• The inflammatory mediators attract and activate
neutrophils and monocytes and stimulate
proliferation of mesangial and endothilial cells.
This results in Hypercellular Glomerulus.
GROSS
MORPHOLOGY
THE KIDNEYS ARE
SYMMETRICALLY
ENLARGED, WEIGHING
UPTO TWICE THE NORMAL
WEIGHT.

THE CORTICAL AS WELL AS

SECTIONED SURFACE
SHOW PETECHIAL
HEMORRHAGES GIVING
THE CHARACTERISTIC
APPEARANCE OF FLEA
BITTEN KIDNEY.
 MICROSCOPY
• ENLARGED HYPERCELLULAR
GLOMERULUS : INFILTRATION
BY LEUKOCYTES,
PROLIFERATION OF
ENDOTHELIAL CELLS
• OBLITERATION OF
GLOMERULAR CAPILLARY
LUMEN
• TUBULES MAY CONTAIN RED
CELL CASTS
• UNREMARKABLE BLOOD
VESSELS.
TYPICAL ELECTRON DENSE IMMUNOFLUORESCENT STAIN
SUBEPITHELIAL “HUMP” SHOWING COARSELY
GRANULAR(LUMPY-BUMPY)
DEPOSITS OF COMPLEMENT PROTEIN
C3
 RAPIDLY PROGRESSIVE
GLOMERULONEPHRITIS/CRESCENTIC GN

• RPGN is a clinical syndrome associated with

severe glomerular injury, but it does not
denote a specific etiology.
• It is characterized by relatively rapid and
progressive loss of renal function associated
with severe oliguria and signs of nephritic
syndrome.
 PATHOGENESIS
• RPGN IS A MANIFESTATION OF DIFFERENT DISEASES
AND NO SINGLE MECHANISM CAN EXPLAIN ALL CASES.
• IN MOST CASES THE GLOMERULAR INJURY IS
IMMUNOLOGICALLY MEDIATED.
• A PRACTICAL CLASSIFICATION DIVIDES RPGN INTO 3
GROUPS ON THE BASIS OF IMMUNOLOGIC FINDINGS:
I.E. THREE SERUM MARKERS,
1) Serum C3 level
2) Anti-GBM antibody
3) Anti –neutrophil cytoplasmic antibody(ANCA)
TYPE 1 ( ANTI-GBM ANTIBODY MEDIATED DISEASE)
- Characterized by linear deposits of IgG
- In some patients the anti-GBM antibodies cross react
with pulmonary basement membrane to produce the
Goodpasture syndrome.
TYPE 2 ( IMMUNE COMPLEX DISEASE)
- Characterized by immune complex deposition
- Complication of poststreptococcal GN
TYPE 3 ( PAUCI IMMUNE GN)
- Defined by the lack of detectable anti-GBM antibodies
or immune complexes.
- Most patients with type 3 RPGN have circulating
ANCAs.
GROSS
MORPHOLOGY
KIDNEYS ARE ENLARGED
AND PALE

PETECHIAL
HEMORRHAGES ON THE
CORTICAL SURFACES
 MICROSCOPY
PRESENCE OF
CRESCENTS IN MOST
OF THE GLOMERULI.
HENCE RPGN IS ALSO
CALLED AS
CRESCENTIC
GLOMERULONEPHRI
TIS

FIBRIN STRANDS ARE

FREQUENTLY
PROMINENT
BETWEEN THE
CELLULAR LAYERS IN
THE CRESCENTS
 MCQS
Q.1) A young child abruptly develops malaise, fever, nausea,
oliguria, and hematuria 1 to 2 weeks after recovery from a
sore throat. The patient have dysmorphic red cell casts in
the urine, mild protienuria, periorbital edema and mild
hypertension.
Which of the following is the most likely cause?

A) Rapidly progressive GN
B) Crescentic GN
C) Post Streptococcal GN
D) None of the above
Q.2) The adjacent
immunofluorescent stain
shows strong linear
staining of IgG along the
Glomerular Basement
Membrane in a patient
presenting with
Goodpasture syndrome.
This PATTERN of staining is
seen in which of the
following types of RPGN?

A) TYPE 1
B) TYPE 2
C) TYPE 3
D) NONE OF THE ABOVE
Q.3) Which of these does not cause crescentic
glomerulonephritis?

A) Rapidly progressive glomerulonephritis

B) Alport syndrome
C) Goodpastures syndrome
D) Henoch Schonlein purpura
Q.4) The prognosis of rapidly progressive
glomerulonephritis(Crescentic GN) depends
upon:
A) Number of crescents
B) Size of crescents
C) Shape of crescents
D) Cellularity of crescents
Q.5) True about post streptococcal
glomerulonephritis is :

A) Linear deposition
B) Diffuse involvement
C) Tram track appearance
D) Global sclerosis