Vasculitis Flashcards
What are the two large vessel vasculites?
- temporal giant cell arteritis
2. Takayasu arteritis
How are takayasu and temporal giant cell arteritis primarily distinguished?
takayasu 50
Temporal giant cell arteritis has what distinguishing symptom?
- major headache
- temporal artery is very tender
What is the pathology behind temporal giant cell arteritis?
T cell mediated immune response against endothelial wall antigen
What is the treatment for temporal giant cell arteritis? why is early treatment important?
corticosteroids
-patients can go blind if not treated ASAP
What vessels does takayasu mainly affect?
aortic arch and branch points
What is a main symptom of takayasu?
pulselessness of extremities
What is the treatment for takayasu?
corticosteroids
What are the three medium vessel vasculites?
- PAN
- kawasaki
- thromboangitis obliterans or buerger
PAN involves many organs, but what organ does it commonly spare?
the lungs
What two underlying conditions is PAN associated with?
Hep B and hairy cell leukemia
In what medium vasculites, is hypertension a common symptom?
PAN
Describe the visual appearance of PAN
“string of pearls”
fibrinoid necrosis with lesions of different ages
What age group and ethnicity does kawasaki typically affect?
asian children<4
What is the main symptom of kawasaki disease?
erythematous rash of palms and soles
Why is kawasaki disease so dangerous?
coronary artery involvement is common
What antibodies are present in kawasaki?
anti-endothelial
What is the treatment for PAN?
corticosteroids and cyclophosphamide
What is the treatment for kawasaki?
aspirin and IVIG
-disease is self-limited
In which vasculites is the ESR typically increased?
- temporal giant cell
- takayasu
What is the visual appearance of buerger disease?
necrotizing vasculitis of the digits
What is buerger disease highly associated with?
smoking
What are the four small artery vasculites?
- Granulomatosis with polyangitis (wegener)
- microscopic polyangitis
- churg-strauss
- henoch schonlein
What three organs are typically affected in granulomatosis wtih polyangitis?
nasopharynx, lungs, kidneys
What is the mechanism behind granulomatosis with polyangitis?
C-ANCA antibodies present - t cell mediated hypersensitivity response
What is the treatment for granulomatosis with polyangitis
cyclophosphamide and corticosteroids
In which vasculites are pANCA antibodies present?
microscopic polyangitis and churg strauss
Microscopic polyangitis typically affects many organs, but which two especially?
lung and kidneys
Eosinophilia and asthma are associated with which vasculites?
churg-strauss
What is the mechanism behind HSP?
IgA immune complex deposition
What population does HSP typically affect?
kids under 20 with a upper respiratory tract infection
