Rapidly Progressive Glomerulonephritis Flashcards
What is RPGN?
Glomerular haematuria (RBC casts or dysmorphic RBCs) and rapidly developing kidney injury
What does histology show for RPGN?
Crescent-shaped cell proliferation in bowman’s capsule
What is Goodpasture’s Disease?
Autoantibodies to type IV collagen
- Attacks glomerulus and pulmonary basement membranes
What are the features of Goodpasture’s Disease?
- AKI
- Haemoptysis
What does immunofluorescence show for Goodpasture’s Disease?
linear IgG deposits along basement membrane
What is the management of Goodpasture’s Disease?
- Plasma exchange (plasmapheresis)
- Steroids,
- Cyclophosphamide
What is immune-complex mediated glomerulonephritis?
- Post-streptococcal glomerulonephritis
- SLE
- IgA nephropathy
- HSP vasculitis
What does immunofluorescence show for immune-complex mediated glomerulonephritis?
Immune complex deposition in subendothelium
What is Pauci-immune glomerulonephritis?
ANCA Vasculitis-associated glomerulonephritis?
What causes Pauci-immune glomerulonephritis?
- Granulomatosis with polyangiitis (Wegner’s granulomatosis)
- Microscopic polyangiitis
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome )
What can you test for Pauci-immune glomerulonephritis?
C-ANCA
Granulomatosis with polyangiitis
P-ANCA
Microscopic polyangiitis
P-ANCA + eosinophilia
Eosinophilic granulomatosis with polyangiitis
What is the pathophysiology of RPGN?
Inflammatory mediators, inflammatory cells, plasma proteins and fibrin go into bowman space. Parietal epithelial cells proliferate due to this causing a crescent moon shape.
Kidneys lose ability to filter blood = low GFR -> acute renal failure