DR. ROOPAM JAIN PROFESSOR & HOD

1. The KIDNEY Rapidly Progressive
Glomerulonephritis
DR. ROOPAM JAIN
PROFESSOR & HEAD, PATHOLOGY

2. Rapidly Progressive Glomerulonephritis
(Synonyms: RPGN, Crescentic GN,
Extracapillary GN)

3. RPGN
• RPGN presents with an acute reduction in renal function resulting in
acute renal failure in a few weeks or months.
• It is characterised by formation of ‘crescents’ (crescentic GN) outside the
glomerular capillaries (extracapillary GN)
• RPGN occurs most frequently in adults
• slight male preponderance.
• Prognosis of RPGN in general is dismal.

4. RPGN - ETIOPATHOGENESIS
• A number of primary glomerular and systemic diseases are characterised
by formation of crescents.
• Based on the etiologic agents and pathogenetic mechanism, patients
with RPGN are divided into 3 groups (Table):
• 1. RPGN in systemic diseases (anti-GBM type)
• 2. Post-infectious RPGN (immune-complex type)
• 3. Pauci-immune RPGN.
• Following three serologic markers are used for categorising RPGN:
• i) serum C3 level,
• ii) anti-GBM antibody; and
• iii) anti-neutrophil cytoplasmic antibody (ANCA)

5. Distinguishing features of 3 main
categories of RPGN

7. RPGN (post-infectious type),
Light microscopic appearance.
There are crescents in Bowman’s space forming adhesions between
the glomerular tuft and Bowman’s capsule.
The tuft shows hypercellularity & leucocytic infiltration.

8. RPGN
diagrammatic representation of ultrastructure of a portion of glomerular
lobule showing epithelial crescent formation & subepithelial granular
deposits.

9. RPGN CLINICAL FEATURES
• Generally, the features of postinfectious RPGN are similar to those of
acute GN, presenting as acute renal failure.
• The patients of Goodpasture’s syndrome may present as acute renal
failure and/or associated intrapulmonary haemorrhage
producing recurrent haemoptysis.
• Prognosis of all forms of RPGN is poor.
• However, post-infectious cases have somewhat better outcome and
may show recovery.