Fogo, Ch. 9 - Crescentic glomerulonephritis & vasculitis Flashcards
What are the three categories of crescentic glomerulonephritis?
Pauci-immune
Immune complex
Anti-GBM
Recall some large and medium-vessel vasculitides.
Large: Takayasu and giant cell arteritis
Medium: Polyarteritis nodosa and kawasaki disease
Recall the ANCA-associated small vessel vasculitides.
Microscopic polyangiitis (Necrotizing vasculitis without granulomas. Pulmonary/kidney)
Granulomatosis with polyangiitis (Necrotizing granulomatous involving respiratory tract and kidney)
Churg-strauss (Eosinophil-rich and necrotizing granulomas)
Recall the immune complex vasculitides.
Anti-GBM disease (Lung and kidney, basement membrane deposition and autoantibodies)
Cryoglobulinemic vasculitis (Cryoglobulin immune deposits, affects skin/kidneys/nerves)
IgA vasculitis (IgA1-dominant deposits in skin/gut)
Anti-C1q vasculitis (Urticaria, hypocomplementemia)
What is anti-GBM disease? Goodpasture’s?
Anti-GBM disease is a small vessel vasculitis that affects glomerular capillaries. Goodpasture’s also has pulmonary alveolar capillary involvement.
What are the LM findings in Anti-GBM disease?
Segmental to global fibrinoid necrosis with crescent formation. Neutrophil infiltration and occasional giant cells. Later sclerosis and fibrous crescents.
What are the IF/EM findings in anti-GBM disease?
Linear IgG/C3 staining along GBM.
No immune complex deposits by EM. Crescents contain fibrin tactoid strands.
How should patients with anti-GBM disease be managed?
Test for ANCA, as many also have it
High-dose cytotoxic agents and plasma exchange
What are the LM findings in pauci-immune glomerulonephritis?
Fibrinoid necrosis and crescent formation, rarely with necrotizing arteritis. Generally cannot be distinguished frm anti-GBM disease by LM.
What are the IF/EM findings in pauci-immune glomerulonephritis?
No or low-intensity (<2) immunoglobulin staining with irregular staining for fibrin. Can have some deposits on EM and GBM breaks.
Recall the features of microscopic polyangiitis.
90% have glomerulonephritis. Few/no immune deposits. Variable necrotizing arteritis.
Recall the features of granulomatosis with polyangiitis.
Granulomatous necrotizing vasculitis affecting small to medium vessels of the kidney and respiratory tract.
Recall the features of Churg-Strauss syndrome.
Eosinophil-rich granulomatous and necrotizing vasculitis affecting small to medium-sized vessels. Associated with asthma and blood eosinophilia. Histologically similar to Wegener’s.
What patterns of ANCA expression are seen in GPA/EGPA?
PR3-ANCA in GPA
MPO-ANCA in EGPA (more common if kidneys are involved)
How are the crescentic glomerulonephritides managed?
High-dose corticosteroids and immunosuppressive agents. Usually good response…
