Glomerular disease - Corbett Flashcards
crescent formation
fibrin deposition is a major inciting effect
(crescents are CONSEQUENCE, not cause)
RPGN major cause
vasculitis (esp the small vessel ones that hit the kidneys too)
immune complex small vessel vasculitis
ANCA-assoc small vessel vasculitis
small vessel vasculitides
- ANCA-associated
- microscopic polyangiitis
- granulomatosis with polyangiitis
- eosinophilic granulomatosis with polyangitiis
- ANCA + but assoc with allergy
ANCAs
antibodies directed against neutrophil cytoplasmic proteins expressed on PMN survace
1. anti-proteinase-3 (aka cANCA, PR3-ANCA)
- PMN azurophilic granule const sharing homology with numerous microbial peptides
2. anti-myeloperoxidase (aka pANCA, MPO-ANCA)
- lysosomal granule constituent involved in free rad gen
GREAT SUMMARY TABLE FOR GLOM DISEASE LEARN THIS
granulomatosis with polyangiitis
keys
mean age 40, M>F
three characteristic features
- necrotizing granulomas
- upper resp tract
- lower resp tract
- necrotizing or granulomatous vasculitis
- affects caps, venules, arterioles, arteries
- prominent in lungs, upper airways
- focal necrotizing glomerulonephritis
PR3 ANCA positive (cANCA, anti-proteinase 3)
- upper and lower resp tract
- nasal involvement, “saddle nose deformity”
- CXR: nodules/cavitation, “ground glass”
- kidney: RPGN
- asymp hematuria
- few to no ICs
microscopic polyangiitis
similar to GPA but NO GRANULOMAS, NO UPPER RESP TRACT INVOLVEMENT
- affects CAPILLARIES, small arterioles, venules
- all lesions of same age, and more widely distributed
- sebmental fibrinoid necrosis of media but no granulomas
clinical findings
- hematuria
- peripheral neuritis (mononeuritis multiplex)
- due to vasculitis of vasa nervorum
MPO-ANCA positive
Henoch-Schonlein purpura
IgA vasculitis
most common form of systemic vasculitis in children
palpable purpura
-
leukocytoclastic vasculitis w or wo fibrinoid necrosis
- vessel damage caused by nuclear debris from infiltrating neutrophils
-
perivascular IgA deposition in almost all patients
- mesangium of kidneys
- cutaneous blood vessels
young individuals (3-15, M>F)
50% of cases preceded by viral URI
classic tetrad
- PAPLABLE PURPURA w/o thrombocytopenia or coag
- arthritis/arthralgia
- abd pain w GI bleed
- renal disease
small vessel disease
summary1
small vessel disease
MUST KNOW for this exam
