3. RPGN
âą RPGN presents with an acute reduction in renal function resulting in
acute renal failure in a few weeks or months.
âą It is characterised by formation of âcrescentsâ (crescentic GN) outside the
glomerular capillaries (extracapillary GN)
âą RPGN occurs most frequently in adults
âą slight male preponderance.
âą Prognosis of RPGN in general is dismal.
4. RPGN - ETIOPATHOGENESIS
âą A number of primary glomerular and systemic diseases are characterised
by formation of crescents.
âą Based on the etiologic agents and pathogenetic mechanism, patients
with RPGN are divided into 3 groups (Table):
âą 1. RPGN in systemic diseases (anti-GBM type)
âą 2. Post-infectious RPGN (immune-complex type)
âą 3. Pauci-immune RPGN.
âą Following three serologic markers are used for categorising RPGN:
âą i) serum C3 level,
âą ii) anti-GBM antibody; and
âą iii) anti-neutrophil cytoplasmic antibody (ANCA)
7. RPGN (post-infectious type),
Light microscopic appearance.
There are crescents in Bowmanâs space forming adhesions between
the glomerular tuft and Bowmanâs 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
âą Generally, the features of postinfectious RPGN are similar to those of
acute GN, presenting as acute renal failure.
âą The patients of Goodpastureâs syndrome may present as acute renal
failure and/or associated intrapulmonary haemorrhage
producing recurrent haemoptysis.
âą Prognosis of all forms of RPGN is poor.
âą However, post-infectious cases have somewhat better outcome and
may show recovery.