blood vessels

Question 1

what is raynauds

Answer 1

condition where spasms of the arteries cause episode of reduced blood flow

Question 2

what is the cause of primary raynauds, who commonly gets it

Answer 2

teenagers

it is idiopathic with no underlying disease

Question 3

what is the cause of secondary raynauds, who commonly gets it

Answer 3

older people

underlying autoimmune disease, ulcers, gangrene

Question 4

what is the treatment of raynauds

Answer 4

keep warm

vasodilators

Question 5

what vasodilators are used in raynauds

Answer 5

Ca2+ channel blockers

PDE5 inhibitors e.g. sildenafil

Question 6

what would the treatment for a digital ulcer causing raynauds

Answer 6

prostacyclin analogue e.g. iloprost

Question 7

what is vasculitis

Answer 7

inflammation of blood vessel s

Question 8

what can vasculitis result in

Answer 8

vessel wall thickening, stenosis, and occlusion with subsequent ischaemia

Question 9

what are the types of vasculitis

Answer 9

large vessel
medium vessel
small vessel
ANCA-assocaited small vessel

Question 10

what are the types of large vessel vasculitis

Answer 10

takayasu arteritis

Giant cell arteritis

Question 11

what are the types of medium vessel vasculitis

Answer 11

polyarteritis nodosa

Kawasaki disease

Question 12

what are the 4 types of ANCA associated vasculitis

Answer 12

granulomatous with polyangitis (aka Wegeners)
Eosinophilic granulomatosis with polyangiitis (aka Churg-strauss)
microscopic polyangitis
renal limited vasculitis

Question 13

what is large vessel vasculitis

Answer 13

applies to primary vasculitis which causes chronic granulomatous inflammation predominately of the aorta and its greater branches

Question 14

who does Takayasua arteritis affect

Answer 14

young women, 20-30

higher rates in asia

Question 15

what is the most common type of systemic vasculitis in adults

Answer 15

Giant cell arteritis

Question 16

what is the histology of Giant cell arteritis

Answer 16

transmural inflammation of the intimate, media, and advent of the affected arteries
PATCHY infiltration by lymphocytes, macrophages, and multinucleate giant cells
vessel wall thickening which can result in arterial lumina narrowing causing distal ischemia

Question 17

what are the symptoms of Giant cell arteritis

Answer 17

visual disturbances (loss, blurring)
continuous headache with focal tenderness
jaw claudication
scalp tenderness
temporal artery may be thickened and tender to touch, non-pulsatile
systemic manifestations (fatigue etc)

Question 18

what investigations should be done in Giant cell arteritis

Answer 18

raised inflammatory markers

temporal artery biopsy ASAP

Question 19

what is the treatment of Giant cell arteritis, when should it be started

Answer 19

novisual impairment = 40mg prednisolone
visual impairment = 60mg prednisolone

start as soon as suspected, don’t wait fir biopsy

Question 20

what is henoch-schonlein purpura

Answer 20

this in an acute, IgA mediated disorder characterised by generalised vasculitis involving the small vessels of the skin, GI tract, kidneys, joints and rarely the lungs and CNS

Question 21

what are the symptoms of henoch-schonlein purpura

Answer 21

URI predates symptoms by a few weeks
purpuric rash over buttocks and lower limbs
abdominal pain
vomiting
joint pain

Question 22

what is the treatment for henoch-schonlein purpura

Answer 22

self-limiting

settles over the course of a few weeks to months

Question 23

what can occur in microscopic polyangiitis

Answer 23

glomerulonephritis (occurs in 90% of patients)

Question 24

what ENT symptoms are associated with granulomatous with polyangitis (aka Wegeners)

Answer 24

nose bleeds
deafness
recurrent sinusitis
nasal crusting 
mouth ulcers 
collapse of nose over time

Question 25

what non-ENT symptoms are associated with granulomatous with polyangitis (aka Wegeners)

Answer 25

purpuric, non-blanching rash most obvious on gravity dependent rash
formation of granulomas anywhere
haemoptysis
cavitating lung lesions

Question 26

what autoantibodies are specific to granulomatous with polyangitis (aka Wegeners)

Answer 26

proteinase-3 (PR3)

Question 27

what investigations should be done in ACNA positive vasculitis

Answer 27

raised inflammatory markers
autoantibodies (ANCA, MPO, anti-PR3)
low C3 and C4
U&E = renal involvement
urinalysis = renal vasculitis
CXR
biopsy of affected area = god standard

Question 28

what is the treatment of ACNA positive vasculitis

Answer 28

IV steroids + cyclophosphamide until the condition is under control
then regular oral steroids and less intense immunosuppressive drug