2- vasculitis Flashcards
what is vasculitis?
inflammation of blood vessels that often leads to inflammation, ischaemia or necrosis
what are the main 3 subtypes of vasculitis?
- large vessel vasculitis
- medium vessel vasculitis
- small vessel vasculitis
*each of these have specific conditions associated with them
what is primary & secondary vasculitis?
primary = unknown cause
secondary = triggered by infection, drug or toxin or part of other inflammatory condition or cancer
what are 2 main subtypes of large vessel vasculitis?
- takayasu arteritis
- giant cell arteritis
what vessels are usually affected by large vessel vasculitis?
aorta and it’s branches
what are some key points about takayasu arteritis (large vessel vasculitis)? e.g. common presentation, go to investigation
- seen in under 40
- more in asian population
- claudication in arms (no pulse) or difference in pulse/blood pressure
- would hear vascular bruit (heard with stethoscope in vessels)
- investigate with CT angiogram
what are some key points about giant cell arteritis (large vessel vasculitis)? e.g. common presentation
- over 50
- typically in temporal artery
- headache, scalp tenderness, visual disturbances, reduced pulsation
what is management of large vessel vasculitis?
steroids (40-60mg prednisolone)
- usually steroids is enough but can add DMARD for takayasu or tocilizumab for resistant giant cell arteritis
what is a type of medium vessel vasculitis?
polyarteritis nodusa
what are some common presenting symptoms for polyarteritis nodusa?
- renal (as often secondary to hep B&C) is most common area to be affected. often hypertension & renal function impairment
- also skin signs like purpura (purple rash) or ulcers or muscle pain
- neurological signs like shooting pains & tingling
- GI signs
what would angiogram show on polyarterium nodusa?
lumps in arteries that looks like nodes (explains name)
what is treatment for polyarteritis nodusa?
steroids!! (main one for these)
if organ threatening then also cyclophosphamide
if non organ threatening then can add immune modulators like azathioprine/methotrexate etc
what are 2 main subtypes of small vessel vasculitis?
- ANCA associated vasculitis
- non-ANCA vasculitis, immune complex small vasculitis
what are the 3 main conditions associated with ANCA vasculitis?
- microscopic polyangiitis (MPA)
- granulomatosis with polyangiitis (GPA)
- eosinophilic granulomatosis with polyangiitis (EGPA)
what is ANCA test?
it tests for autoantibodies to diagnose the conditions of ANCA associated small vessel vasculitis
there is p-ANCA test = detects MPO antigen
there is c-ANCA test = detects PR3 antigen
*can remember as c = 3rd alphabet
what small vessel vasculitis ANCA conditions have
a) MPO antigens?
b) PR3 antigens?
a) MPO antigens are in MPA and EGPA (microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis)
b) PR3 antigens are in GPA (granulomatosis polyangiitis)
what is MPA?
microscopic polyangiitis = absent of granulomas. has kidney inflammation & lung bleeding. MPO antigen
what is GPA?
granulomatosis polyangiitis = granuloma formation affecting resp tract & kidneys
what is EGPA?
eosinophilic granulomatosis polyangiitis. involves eosinophils. associated with asthma & allergy
what are tests to diagnose small vessel vasculitis?
- urine dipstick, look for RBCs (hematuria) and proteinuria
- FBC, renal function, LFTs
- inflammatory markers
- ANCA test (immunofluorescence technique)
what are some general clinical features associated with small vessel vasculitis?
- cutaneous = palpable purpura, ulcers, arthritis
- ENT = sinusitis, nasal crusting, mouth ulcers, otitis media & deafness, saddle node (cartilage damage from ischaemia)
- resp = cough, haemoptysis, breathlessness
- ocular = conjunctivitis, uveitis
- renal = necrotising glomerulonephritis
- nervous system problems
what is management of ANCA vasculitis?
organ threatening - glucocorticoids (prednisolone) & rituximab & ciclophsophamide. maintain with rituximab & azathioprine
non organ threatening - same but without cyclophosphamide
what are a couple of points that suggest higher relapse risk?
- GPA more likely to relapse over MPA (because PR3-ANCA more severe disease)
- if persistent ANCA +ve even after treatment
what is type of non ANCA vasculitis?
henoch-schonlein purpura (HSP) = acute IgA mediated disorder
