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RPGN Types Pathology
1. Renal Pathology
for medical students
Rapidly Progressive
Glomerulonephritis
Professor Wadie M Y Elmadhoun, MBBS, MSc.,MD
E-mail: wadie2222@yahoo.com
Kidney Pathology â RPGN â Prof. Wadie 1
2. Presentation outlines
1. Intended Learning outcomes (ILOs).
2. Rapidly Progressive Glomerulonephritis
(RPGN): definition, pathogenesis and clinical
course.
3. Types of RPGN: Type I, Type II, Type III.
5. Quiz.
6. Further learning resources.
Kidney Pathology â RPGN â Prof. Wadie
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3. Intended Learning outcomes (ILOs)
⢠By the end of this session, the learner should be
able to:
1. Explain what is meant by Rapidly Progress GN,
discuss its nature, clarify its clinical course,
causes and pathogenesis.
2. Differentiate between the various types of
RPGN.
Kidney Pathology âRPGN â Prof. Wadie
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4. Definition of the Rapidly Progressive
Glomerulonephritis (RPGN)
⢠A clinical syndrome characterized by:
⢠Rapid loss of renal function: more than 50%
decline of GFR within 3 months.
⢠Crescent-shaped scars in most glomeruli,
⢠If not treated, will progress to acute renal
failure and death within months.
Kidney Pathology â RPGN â Prof. Wadie 4
5. Crescents in 2 glomeruli
Kidney Pathology â RPGN â Prof. Wadie 5
6. Crescent shape in glomerulus
Kidney Pathology â RPGN â Prof. Wadie 6
8. Overview of RPGN/Crescentic GN
⢠RPGN is a clinical definition, NOT a specific
pathologic GN.
⢠Also known as âCRESCENTIC GNâ
⢠It is caused by either
immunological damage to
glomeruli or associated with anti-
neutrophil cytoplasmic antibody
(ANCA). Kidney Pathology â RPGN â Prof. Wadie 8
9. Rapidly Progressive Glomerulonephritis
(Crescentic GN)
⢠Type I: anti-GBM antibodies.
⢠Type II :immune complex
deposition in GBM.
⢠Type III : No remarkable immune damage,
associated with anti-neutrophil cytoplasmic
antibody (ANCA).
Kidney Pathology â RPGN â Prof. Wadie
10. Signs and symptoms
⢠Most types of RPGN are characterized by:
⢠severe and rapid loss of kidney function
⢠with marked hematuria; red blood cell casts in
the urine; and proteinuria
⢠Some patients also experience hypertension
and edema.
⢠Severe disease is characterized by oliguria or
anuria.
Kidney Pathology â RPGN â Prof. Wadie
11. Two urine samples showing gross and microscopic
hematuria: in RPGN
Kidney Pathology â RPGN â Prof. Wadie
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12. CLASSIFICATION
⢠Crescentic glomerulonephritis (CrGN)
⢠RPGN can be classified into three types, based
upon the pathogenesis and
immunofluorescence patterns:
Kidney Pathology â RPGN â Prof. Wadie 12
13. 1. Type I CrGN
⢠also called anti-GBM antibody Crescentic GN: is
characterized by the presence of autoantibodies
directed against type IV collagen in the (GBM).
⢠Some cases are associated with antibodies
directed against the basement membrane of lung
alveoli, producing Goodpasture syndrome.
⢠Type I accounts for less than 20% of RPGN.
⢠Plasma exchange or plasma-pheresis benefit
patients with type I CrGN, but not types II or III.
Kidney Pathology â RPGN â Prof. Wadie 13
14. Anti-GBM Antibody, type I RPGN
(Linear immunoflourescence in Type I CrGN)
Kidney Pathology â RPGN â Prof. Wadie 14
15. 2. Type II CrGN
⢠Characterized by deposition of immune
complexes in glomerular tissues. (Granular IF)
⢠Any immune complex diseaseâincluding SLE,
acute proliferative glomerulonephritis,
HenochâSchĂśnlein purpura, and IgA
nephropathy may progress to RPGN.
⢠Type II RPGN accounts for about 40% of cases
Kidney Pathology â RPGN â Prof. Wadie 15
18. RPGN type II: secondary to IgA nephropathy
Kidney Pathology â RPGN â Prof. Wadie 18
19. 3. Type III CrGN
⢠Also known as pauci-immune RPGN, and features
neither immune complex deposition nor anti-GBM
antibodies.
⢠Instead, the glomeruli are damaged perhaps
through the activation of neutrophils in response to
ANCA.
⢠Type III RPGN may be (primary, or idiopathic) or
associated with a systemic disease (secondary) to
ANCA-associated vasculitis such as granulomatosis
with polyangiitis.
⢠Type III RPGN accounts for more than 40% of RPGN
Kidney Pathology â RPGN â Prof. Wadie 19
20. Diagnosis of RPGN
⢠Impaired kidney function in an individual who has
had the condition for fewer than three months is
characteristic of RPGN.
1. Renal biopsy: is the most important
investigation. Crescents are identified on
microscopy + linear or granular pattern in
immunofluorescence.
2. Raised serum creatinine and urea.
3. Urine examination: oliguria or anuria, hematuria
and proteinuria.
Kidney Pathology â RPGN â Prof. Wadie 20
22. Diagnosis
⢠Serum analysis often aids in the diagnosis of a
specific underlying disease.
1. The presence of anti-glomerular basement
membrane (GBM) antibodies suggests type I
RPGN.
2. Antinuclear antibodies (ANA) may support a
diagnosis of systemic lupus erythematosus and
type II RPGN;
3. Type III is associated with anti-neutrophil
cytoplasmic antibodies (ANCA)-positive serum.
Kidney Pathology â RPGN â Prof. Wadie 22
25. ASSIGNMENT
â˘You are asked to revise
the next slide carefully,
then write a two-page
essay about its topic.
Kidney Pathology â RPGN â Prof. Wadie
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