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19 Cards in this Set
- Front
- Back
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What is vasculitis?
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A heterogeneous group of immune mediated disorders that cause inflammation and damage to blood vessel walls leading to tissue ischemia and organ failure
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What are the main steps in vasculitis pathogenesis?
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1. Leukocyte infiltration or immune complex deposition
2. Inflammatory cascade/damage 3. Lumenal Occlusion 4. Tissue Necrosis 5. Organ Failure |
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How is the type of vascultis classified?
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By vessel size
1. Large Vessel Vascultis 2. Medium Vessel Vasculitis 3. Small Vessel Vasculitis (either ANCA or immune complex) |
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What 2 tests can be run to provide a definitive diagnosis of vasculitis?
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1. Biopsy
2. Angiogram |
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What are the 3 cardinal histological features of vascultis?
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1. Leukocyte Infiltration
2. Damage to vessel wall 3. Immune complex deposition |
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What is the most important medium vessel vasculitis?
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Polyarteritis nodosum (PAN)
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What does PAN spare?
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Lungs and glomeruli (microcirculation)
- can affect kidney but spares the glomeruli |
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On an angiogram, what are the 3 classical findings of PAN?
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1. "Beads on a string" - widening/narrowing of vessel
2. Narrowing/stenosis 3. Aneurysm |
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Digital Gangrene is seen in what condition?
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PAN
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Are immune complexes and/or ANCAs present in PAN?
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NO, only leukocyte infiltration
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What are ANCAs and what are they associated with?
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- Anti-Neutrophil cytoplasm antibodies (recognize antigens in cytoplasmic granules of neutrophils)
- Associated with ANCA Associated Vasculitis (AAV) |
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What are the patterns of ANCA?
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1. C-ANCA (cytoplasmic staining)
2. P-ANCA (perinuclear staining) - recognizes myeloperoxidase (MPO) |
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What kidney symptom is often seen with ANCA-Associated Vasculitis (AAV)?
What shape? |
Glomerulonephritis - see crescent formation in glomeruli
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What sequelae is seen in AAV?
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Edema bc of fluid retention in the kidney
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Pathogenically, was role do ANCAs play in AAV?
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- ANCAs cause neutrophil activation
- Neutrophil activation causes endothelial cell damage in the capillaries |
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What is the most common vasculitis?
How does it present? |
Leukocytoclastic Vasculitis (LCV)
- usually presents as skin rash |
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What immune compound is characteristic of LCV?
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Immune Complex Deposition
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What ratio of antigen:Ab causes immune complexes to form?
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Excess antigen
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What is the pathogenesis of LCV?
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1. Excess antigen causes immune complexes to form
2. Complement binds and release C3a/C5a which attract neutrophils and basophils 3. Activated neutrophils damage endothelial cells and vessel wall |
