Vasculitis Flashcards
How does primary vasculitis arise?
Inflammatory response (can be autoimmune) that occurs in the walls of blood vessels, causing damage and reduced blood flow
List the 2 main large vessel vasculitis
Giant cell arteritis
Takayasu arteritis
List the 2 main medium vessel vasculitis
Polyarteritis nodosa
Kawasaki disease
Small vessel vasculitis can be generally divided into which 2 groups?
ANCA-associated
Non-ANCA-associated
Takayasu arteritis is commoner in males and in Asian populations. True/False?
False
Commoner in females and Asian populations
Giant cell arteritis affects those over 50 and is associated with which other disease?
Polymyalgia rheumatica
What is the most common finding in giant cell arteritis?
Carotid bruit
What is the mainstay of treatment for large vessel vasculitis?
Steroid (40-60mg prednisolone)
Which group of people is Kawasaki disease seen in?
Children
Which part of the artery is usually affected in polyarteritis nodosa?
Vessel bifurcations
Which hepatitis is associated with polyarteritis nodosa?
Hepatitis B
What are the old names for the ANCA-positive small vessel vasculitic diseases?
Wegener’s granulomatosis
Churg-Strauss syndrome
Microscopic polyangitis
What are the new names for the ANCA-positive small vessel vasculitic diseases?
Granulomatosis with polyangitis (GPA)
Eosinophilic granulomatosis with polyangitis (EGPA)
Microscopic polyangitis (MPA)
Which of the 3 main ANCA-positive small vessel vasculitic diseases involve granulomatous inflammation?
GPA
EGPA
Which ANCA-positive small vessel vasculitic disease is associated with asthma?
EGPA
Which ANCA-positive small vessel vasculitic diseases are associated with glomerulonephritis?
GPA
MPA
GPA is more common in males. True/False?
True
When does GPA typically present?
35-55yrs old
What effect does GPA have on the nose?
Saddle nose due to cartilage ischaemia
What is the usual cutaneous manifestation of GPA?
Palpable purpura
What is mononeuritis multiplex and what effect can it have in GPA?
Impaired blood supply to nerve
Wrist/foot drop
What is the main difference between GPA and EGPA?
Late onset asthma
High eosinophil count
What are the 2 main types of ANCA?
pANCA
cANCA
Which vasculitis is associated with pANCA?
MPA
EGPA
Which vasculitis is associated with cANCA?
GPA
What is the antigen that pANCA primarily responds to in MPA?
MPO
What is the antigen that cANCA primarily responds to in GPA?
PR3
Outline treatment for localised/early systemic AAV
Methotrexate + steroid
Outline treatment for generalised/systemic AAV
Cyclophosphamide + steroid
Ritixumab + steroid
Azathioprine
Outline treatment for refractory AAV
IV immunoglobulins
Rituximab
What is the main non-ANCA-associated small vessel vasculitic disease?
Henoch-Schonlein Purpura (HSP)
Which Ig mediates HSP?
IgA
HSP is most common in which age range?
2-11 yrs old
