Vasculitis Flashcards
What is vasculitis?
Inflammation of blood vessels, with ischaemia, necrosis and organ inflammation
True or False
Vasculitis only affects arteries and veins
False.
Venules, arterioles and capillaries can all be affected too
What are the two types of vasculitis?
Primary and secondary
What can trigger secondary vasculitis?
Infection, drugs or a toxin
What is the pathogenesis of vasculitis?
Something causes dendritic cells to release cytokines which causes a cascade of WBC infiltration which leads to endothelial damage
How is vasculitis classified?
Large vessel,
medium vessel
ANCA-associated small vessel
immune complex small vessel
What is the classical presentation of vasculitis?
Depends very much on where it affects.
Fever, malaise, fatigue and weight loss are all common,
What are the common causes of large vessel vasculitis?
Giant cell arteritis
Takaysu arteritis
What condition has a link with giant cell arteritis?
Polymyalgia rheumatica
Who is more likely to develop Takaysu arteritis?
Females under 40.
More common in Asians
Where does giant cell arteritis affect?
Typically temporal arteries but can also be in aorta and other great vessels
What clinical findings may be present in large vessel vasculitis?
Bruit, especially at carotids
Blood pressure different at extremities
Claudication
Hypertension
What are the classical symptoms of temporal arteritis?
Jaw claudication
Unilateral, temporal headache
Prominent, non-pulsating temporal arteries.
What is the major concern with temporal arteritis?
risk of blindess due to ischaemia of optic nerve
What investigations would you do if you suspected TA?
Inflammatory markers -ESR, CRP, PV
MR angiogram
True or False
Temporal artery biopsy is the definitive investigation
False.
Skip lesions occur so a negative biopsy does not rule out TA
What is the management of TA?
40mg prednisolone
DMARDs if necessary
What is Kawasaki disease?
Medium vessel vasculitis
Kids
What is polyarteritis nodosa?
Medium vessel vasculitis
necrotising inflammatory lesion at bifurcations
Affects skin, gut and kidneys
What is granulomatois with polyangitis (GPA)?
Granulomatous inflammation of respiratory tract and sm. & med. vessels.
Glomerulonephritis common.
What did GPA used to be called?
Wegner’s syndrome
Who is GPA most common in?
Slightly more common in males. 35-55
What are the features of GPA?
Constitutional symptoms and arthralgia
Lots of ENT features- sinusitis, ulcers, saddle nose, nosebleeds
Also conjunctivitis, uveitis and deafness
What is the medical term for nosebleeds?
Epistaxis
What respiratory features are present in GPA?
Cough, haemoptysis, pulmonary infiltrates
What is the difference in GPA and EGPA?
EGPA is eosinophilic and presents with asthma
What did EGPA used to be called?
Churg-Strauss
If you suspected a (E)GPA what investigations would you do?
Inflammatory markers
CXR
ANCA
Urinalysis
What is the treatment cascade for (E)GPA?
Methotrexate &; steroids if localised
Cyclophosphamide &; steroids if systemic
IV immunoglobulin if progressive and unresponsive
What is Henoch-Schonlein purpura?
Acute IgA mediated disorder
Who normally gets Henoch- Schonlein purpura?
Majority in children 2-11
Where does Henoch- Schonlein purpura normally affect?
Small vessels of skin, GI tract, kidneys and joints.
What normally comes before HSP?
URTI, GI infection.
Normally group A strep.
What does HSP present as?
Purpuric rash over buttocks and lower limbs
Colicky abdo pain
Bloody diarrhoea
Joint pain
What is the management for HSP?
resolves in 8 weeks
urinalysis essential to screen for renal involvement
How can skin present in GPA?
Palpable, non-blanching purpuras
What renal involvement can develop in GPA?
Necrotising glomerulonephritis so do urinalysis
What is ANCA?
Ab against proteins in the cytoplasmic against neutrophils
What are the two types of ANCA?
cANCA
pANCA
Which condition is associated with cANCA?
GPA
Which ANCA is PR3 associated with?
cANCA
Which ANCA is MPO associated with?
pANCA
