2- vasculitis

Question 1

what is vasculitis?

Answer 1

inflammation of blood vessels that often leads to inflammation, ischaemia or necrosis

Question 2

what are the main 3 subtypes of vasculitis?

Answer 2

1. large vessel vasculitis
2. medium vessel vasculitis
3. small vessel vasculitis
   *each of these have specific conditions associated with them

Question 3

what is primary & secondary vasculitis?

Answer 3

primary = unknown cause
secondary = triggered by infection, drug or toxin or part of other inflammatory condition or cancer

Question 4

what are 2 main subtypes of large vessel vasculitis?

Answer 4

1. takayasu arteritis
2. giant cell arteritis

Question 5

what vessels are usually affected by large vessel vasculitis?

Answer 5

aorta and it’s branches

Question 6

what are some key points about takayasu arteritis (large vessel vasculitis)? e.g. common presentation, go to investigation

Answer 6

• 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

Question 7

what are some key points about giant cell arteritis (large vessel vasculitis)? e.g. common presentation

Answer 7

• over 50
• typically in temporal artery
• headache, scalp tenderness, visual disturbances, reduced pulsation

Question 8

what is management of large vessel vasculitis?

Answer 8

steroids (40-60mg prednisolone)

• usually steroids is enough but can add DMARD for takayasu or tocilizumab for resistant giant cell arteritis

Question 9

what is a type of medium vessel vasculitis?

Answer 9

polyarteritis nodusa

Question 10

what are some common presenting symptoms for polyarteritis nodusa?

Answer 10

• 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

Question 11

what would angiogram show on polyarterium nodusa?

Answer 11

lumps in arteries that looks like nodes (explains name)

Question 12

what is treatment for polyarteritis nodusa?

Answer 12

steroids!! (main one for these)

if organ threatening then also cyclophosphamide
if non organ threatening then can add immune modulators like azathioprine/methotrexate etc

Question 13

what are 2 main subtypes of small vessel vasculitis?

Answer 13

1. ANCA associated vasculitis
2. non-ANCA vasculitis, immune complex small vasculitis

Question 14

what are the 3 main conditions associated with ANCA vasculitis?

Answer 14

1. microscopic polyangiitis (MPA)
2. granulomatosis with polyangiitis (GPA)
3. eosinophilic granulomatosis with polyangiitis (EGPA)

Question 15

what is ANCA test?

Answer 15

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

Question 16

what small vessel vasculitis ANCA conditions have
a) MPO antigens?
b) PR3 antigens?

Answer 16

a) MPO antigens are in MPA and EGPA (microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis)
b) PR3 antigens are in GPA (granulomatosis polyangiitis)

Question 17

what is MPA?

Answer 17

microscopic polyangiitis = absent of granulomas. has kidney inflammation & lung bleeding. MPO antigen

Question 18

what is GPA?

Answer 18

granulomatosis polyangiitis = granuloma formation affecting resp tract & kidneys

Question 19

what is EGPA?

Answer 19

eosinophilic granulomatosis polyangiitis. involves eosinophils. associated with asthma & allergy

Question 20

what are tests to diagnose small vessel vasculitis?

Answer 20

• urine dipstick, look for RBCs (hematuria) and proteinuria
• FBC, renal function, LFTs
• inflammatory markers
• ANCA test (immunofluorescence technique)

Question 21

what are some general clinical features associated with small vessel vasculitis?

Answer 21

• 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

Question 22

what is management of ANCA vasculitis?

Answer 22

organ threatening - glucocorticoids (prednisolone) & rituximab & ciclophsophamide. maintain with rituximab & azathioprine

non organ threatening - same but without cyclophosphamide

Question 23

what are a couple of points that suggest higher relapse risk?

Answer 23

• GPA more likely to relapse over MPA (because PR3-ANCA more severe disease)
• if persistent ANCA +ve even after treatment

Question 24

what is type of non ANCA vasculitis?

Answer 24

henoch-schonlein purpura (HSP) = acute IgA mediated disorder

Question 25

what is HSP non ANCA small vessel vasculitis?

Answer 25

IgA mediated disorder = generalised vasculitis involving involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system (CNS) - happens in kids after infection like URTI, GI etc (mostly after group A strep)

Question 26

what is treatment of HSP?

Answer 26

self limiting illness if resistant = steroids & immunosuppressive drugs