R: Vasculitis

Question 1

what is vasculitis

Answer 1

umbrella term for inflammation of vessel walls

Question 2

what are the 2 features used to classify vasculitides?

Answer 2

size of blood vessels & presence of anti-neutrophil cytoplasmic antibodies (ANCA) in blood

Question 3

what do we mean by large vessel?

Answer 3

aorta & its tributaries

Question 4

pathophysiology of vasculitides?

Answer 4

dendritic cells activated releasing inflammatory cytokines > t-cells activated promoting granuloma formation & macrophage activation > mediators released causing vascular inflammation, endothelial damage etc

Question 5

Polymyalgia reumatica and giant cell arteritis are a…

Answer 5

large vessel vasculitis

Question 6

what are Polymyalgia reumatica and giant cell arteritis

Answer 6

granulomatous infiltrations of walls of large vessels

- GCA: arteritis of large cerebral arteries

Question 7

s/s of Polymyalgia reumatica and giant cell arteritis

Answer 7

PMR: non-specific: fever, malaise, fatigue, hip/bum/thigh pain and stiffness that is worse in morning

GCA: headaches, tenderness of scalp, jaw claudication, visual loss, tenderness of occipital arteries

Question 8

ix for Polymyalgia reumatica and giant cell arteritis

Answer 8

bloods: inflammatory markers
LFTs: ALP & gamma GT raised
temporal artery biopsy
MR Angio *may show thickened vessel walls)

Question 9

mx of Polymyalgia reumatica and giant cell arteritis

Answer 9

low dose long term steroids + steroid sparing agents (MTX)

Question 10

what is another large vessel vasculitis more common in females and asian population?

Answer 10

Takayasu’s Arteritis

Question 11

what is a medium vessel vasculitides?

Answer 11

polyarteritis nodosa/ Kawasaki’s disease

Question 12

epi for polyarteritis nodosa/ Kawasaki’s disease

Answer 12

PN: middle aged men

Kawasaki’s: children <5

Question 13

aetiology of polyarteritis nodosa/ Kawasaki’s disease

Answer 13

PN: hepB?

Kawasaki’s: infective cause

Question 14

pathophysiology of polyarteritis nodosa/ Kawasaki’s disease

Answer 14

fibrinoid necrosis of vessel walls with micro aneurysm formation, thrombosis, infarction

Question 15

s/s of polyarteritis nodosa/ Kawasaki’s disease

Answer 15

PN: fever/wt loss

• neuro: mononeuritis multiplex
• renal: haematuria/ proteinuria
  cardiac: coronary arteritis

kawasakis: fever >5days, conjunctival congestion 2-4days after onset, dry/red oral cavity, cervical LN

Question 16

ix of polyarteritis nodosa/ Kawasaki’s disease

Answer 16

PN: bloods, biopsy, angio, screen fro hepB, urinalysis
K: fever + 4/5 of other s/s + inc CRP

Question 17

mx for polyarteritis nodosa/ Kawasaki’s disease

Answer 17

PN: corticosteroids + immunosuppression

K: immunoglobulin & aspirin

Question 18

how are small vessel vasculitides divided?

Answer 18

ANCA +Ve & ANCA -ve

Question 19

What are the anca +ve small vessel vasculitides?

Answer 19

granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis

Question 20

T/F: Wegner’s is another name for EGPA?

Answer 20

F: Wegner’s is another name for GPA

Question 21

epi for GPA & EGPA?

Answer 21

GPA: late adulthood
EGPA: early adulthood

Question 22

s/s for GPA & EGPA?

Answer 22

GPA: fever, arthralgia

• ENT*: bloody nasal discharge, nasal discharge, otitis media
• renal: necrotising glomerulonephritis
• resp: cavities on x-ray, cough, pleuritic pain
• cutaneous: non-blanching purpura rash
  neuro: CN palsies, mononeuritis multiplex

EGPA: triad of rhinitis, late onset asthma that is difficult to control, raised peripheral blood eosinophilia

Question 23

ix for GPA/EGPA?

Answer 23

GPA: biopsy*, bloods, urinalysis
- serology: c-ANCA +ve, inc PR3 antibodies

EGPA: biopsy, x-ray for pulmonary infiltrates
- serology: p-ANCA +ve

Question 24

mx for GPA/EGPA?

Answer 24

GPA: glucocorticoids & DMARDs
RGPA: corticosteroids + DMARDs

• DMARDs= stronger given for more severe disease e.g. localised= MTX, generalised= rituximab

Question 25

golemurlonephritis is a common complication of another medium vessel vasculitis, what is this?

Answer 25

microscopic polyangiitis

Question 26

what are the 2 ANCA -ve small vessel vasculitides?

Answer 26

Henoch-Schonlein Purpura and Goodpasture's syndrome

Question 27

what is HSP?

Answer 27

acute IgA mediated vasculitis involving GI, kidneys, joints and skin

Question 28

epi for HSP?

Answer 28

2-11 yo

Question 29

aetiology of HSP?

Answer 29

often follows an URTI, pharyngeal or GI infection

Question 30

what is the causative organism for HSP?

Answer 30

group A streptococci

Question 31

s/s of HSP?

Answer 31

purpuric skin rash on buttocks/ lower limbs, abdominal colic & bloody diarrhoea/ vomiting, glomerulonephritis

Question 32

mx for HSP?

Answer 32

self-liming (8weeks)

Question 33

what is the triad for Goodpasture's syndrome?

Answer 33

pulmonary haemorrhage, glomerulonephritis, antibody presence to basement membrane of kidneys & lungs

Question 34

mx for GPS?

Answer 34

plasmapheresis and immunosuppression

Question 35

what is Behcet's vasculitis?

Answer 35

chronic AI vasculitis which characteristically presents with recurrent oral and genital ulcers

Question 36

T/F: Behcet's is a treatable condition

Answer 36

T but it is a relapsing-remitting condition

Question 37

which gene may be implicated and is a good indicator for severity in Behcet's?

Answer 37

HLA B51

Question 38

s/s of Behcet's affects the whole body- from lungs (pulmonary artery aneurysm) to CNS (memory impairment), but what is the classical triad?

Answer 38

oral ulcers, genital ulcers, uveitis

Question 39

what is the Ix for Behcet's?

Answer 39

pathergy test- wheal >55mm formation with subcutaneous abrasion

Question 40

mx for Behcet's?

Answer 40

combo of steroids & colchicine