Rapidly Progressive Glomerulonephritis - Crescentic Glomerulonephritis - Renal 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 – RPGN – 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 – RPGN – 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 –RPGN – 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.
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5. Crescents in 2 glomeruli
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6. Crescent shape in glomerulus
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7. Crescent in RPGC
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8. Overview of RPGN/Crescentic GN
• RPGN is a clinical definition, NOT a specific
pathologic GN.
• Also known as “CRESCENTIC GN”
• It is caused by either
immunological damage to
glomeruli or associated with anti-
neutrophil cytoplasmic antibody
(ANCA). Kidney Pathology – RPGN – 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 – RPGN – 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 – RPGN – Prof. Wadie

11. Two urine samples showing gross and microscopic
hematuria: in RPGN
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12. CLASSIFICATION
• Crescentic glomerulonephritis (CrGN)
• RPGN can be classified into three types, based
upon the pathogenesis and
immunofluorescence patterns:
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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.
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14. Anti-GBM Antibody, type I RPGN
(Linear immunoflourescence in Type I CrGN)
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15. 2. Type II CrGN
• Characterized by deposition of immune
complexes in glomerular tissues. (Granular IF)
• Any immune complex disease—including SLE,
acute proliferative glomerulonephritis,
Henoch–Schönlein purpura, and IgA
nephropathy may progress to RPGN.
• Type II RPGN accounts for about 40% of cases
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16. Granular IF
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17. Granular pattern
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18. RPGN type II: secondary to IgA nephropathy
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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
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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.
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21. Renal biopsy showing a crescent
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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.
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25. ASSIGNMENT
•You are asked to revise
the next slide carefully,
then write a two-page
essay about its topic.
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26. Kidney Pathology – RPGN – Prof. Wadie 26

27. Quiz 1: what is the pathological change
seen in this microphotograph?
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Answer: crescents.

28. Further sites for learning
• https://en.wikipedia.org/wiki/Rapidly_progres
sive_glomerulonephritis
• https://webpath.med.utah.edu/RENAHTML/R
ENALIDX.html#9
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29. Enjoy the scene
of this crescent.
Thanks
Kidney Pathology – Nephritic Syndrome – Prof. Wadie 29