Vasculitis Flashcards
What are the primary criteria for vasculitis pathology?
- vessel wall necrosis with nuclear fragmentation
- invasion of vessel walls with polymorphonuclear leukocytes
- Fibrinoid deposits in or adjacent to vessel wall
What are the secondary criteria for vasculitis pathology?
- perivascular hemorrhage
- Chronic granulomatous inflammation
- Perivascular fibrosis
- Thrombosis of vessel
What is humoral mediated vasculitis?
Circulating Ab-Ag immune complexes
Auto-antibodies (ANCA, anti-endothelial cell Abs):
- Increased permeability of vessel intima
- Immune complex deposition inflammation
- Complement activation inflammation
- Recruitment, invasion of neutrophils necrosis
- Proinflammatory cytokines (IL-1, IL_6, TNFalpha)
- Endothelial cell injury is thrombogenic clots
What is cell mediated vasculitis?
Lymphocytes and macrophage
- Granulomatous reaction
- Delayed type hypersensitivity (DTH) reaction
What is the difference between primary vasculitis and secondary vasculitis?
Primary:
-idiopathic loss of tolerance to self
Secondary: Response to..... -infection -tumors -immune complexes -toxins
What are the cutaneous manifestations of vasculitis?
- rash
- purpura
- ulceration
- gangrene
Why are there nervous system manifestations in vasculitis?
-infarcts of vasa nervorum of peripheral nerves
What is Takayasu’s disease?
LARGE VESSEL
- Decreased brachial artery pulse
- Upper to lower extremity BP differential >10 mm Hg
- Subclavian or aortic bruit
- Angiogram abnormality
What is hypersensitivity vasculitis?
- Reaction to drug
- Maculopapular rash: flat and raised lesions
- Palpable purpura
- Perivascular granulocytes on skin biopsy
- Joint pains
- Enlarging lymph nodes
How do corticosteroids treat vasculitis?
Binds intracellular hormone receptor
Inhibits cytokine production
Inhibit neutrophil/monocyte phagocytosis, granule release
Inhibit lymphocyte migration
Causes lympholysis
What types of drug agents can we use to treat vasculitis?
- Corticosteroids
- Cytotoxic agents
- B-cell depletion
- TNF blockade
- IL-1 inhibitors
What does the treatment of vasculitis depend on?
the size of the vessels involved
What is Kawasaki’s disease?
MEDIUM VESSELS
- FUO (fever of unknown origin)
- red lips
- bilateral conjunctivits
- Cervical lymphadenopathy
- desquamination of fingers & toes
How does vasculitis cause clinical symptoms?
A. Inflammation leads to endothelial injury and vasospasm that contribute to thrombosis and downstream ischemia.
B. Inflammation of blood vessels leads to neo-angiogenesis and vascular tumors.
C. Cytokine production causes decreased endothelial cell adhesion, leaky vessels, hypovolemic shock, and low blood pressure.
D. Autoantibodies bind to trapped antigens in the vessel wall, leukocytes bind autoantibodies and damage endothelial cells, causing leaky vessels, hypovolemic shock, and low blood pressure.
E. Tissue macrophages infiltrate and proliferate in the media layer of the vascular wall in response to lipid accumulation, leading to plaque build-up, luminal narrowing, and downstream ischemia.
A. Inflammation leads to endothelial injury and vasospasm that contribute to thrombosis and downstream ischemia.
How are forms of vasculitis currently classified?
A. By organ system (kidneys, lungs, brain, etc.)
B. By etiology (immune complex, ischemic, infectious, neoplastic, etc.)
C. By MHC restriction (HLA-DR, HLA-DQ, HLA-B27, etc.)
D. By caliber of the vessels involved
E. By layer of vessel initially involved (intima, media, adventitia)
D. By caliber of the vessels involved
