Vasculitis

Question 1

What is the definition of vasculitis?

Answer 1

Vessel wall inflammation

Question 2

What is the pathophysiology of vasculitis?

Answer 2

Immune complex disease

Antibody dependent cellular cytotoxicity leads to endothelial activation and coagulopathy

Question 3

Give 5 systemic symptoms of vasculitis

Answer 3

Malaise
Fever
Weight loss
Myalgia
Arthralgia
Sweats
Fatigue

Question 4

Give 3 skin changes seen in vasculitis

Answer 4

Purpura
Ulceration
Infarction
Rash

Question 5

Give 3 GI effects of vasculitis

Answer 5

Mouth ulcers
Abdo pain
Diarrhoea

Question 6

Give 5 respiratory signs of vasculitis

Answer 6

Cough
Wheeze
Haemoptysis
Dyspnoea

Question 7

Give 3 ENT signs of vasculitis

Answer 7

Epistaxis
Change of nose shape
Sinusitis
Deafness

Question 8

What investigations would be done in someone where vasculitis is suspected?

Answer 8

Urinalysis
ESR
CRP
WCC
Biopsy 
Angiogram 
Organ specific tests

Question 9

How is vasculitis managed?

Answer 9

Cyclophosphamide and high dose steroids to induce remission

To maintain remission, azathioprine, methotrexate, low dose steroids

Question 10

Give 2 examples of large primary vasculitis

Answer 10

Takavasu’s arteritis
Giant cell arteritis
Polymyalgia rheumatica

Question 11

Give 2 examples of medium primary vasculitis

Answer 11

Polyarteritis nodosa
Kawasaki disease
Isolated CNS vasculitis

Question 12

Give 2 examples of small primary vasculitis

Answer 12

Wegeners granulomatosis (GPA)
EGPA
Microscopic polyangitis

Question 13

Give 4 secondary causes of vasculitis

Answer 13

RA
SLE
Arthritis
Malignancy
Infection 
Drugs- cocaine

Question 14

What common facial deformity is seen in Wegener’s granulomatosis (GPA)

Answer 14

Saddle nose deformity

Question 15

What are the classic triad of systems involved in GPA?

Answer 15

Upper airway –> nasal obstruction, epistaxis, saddle nose

Lungs –> sinusitis, cough, chest pain, pleuritis, stridor

Kidneys –> glomerulonephritis, crescent formation, proteinuria

Question 16

What investigations should be done if GPA is suspected?

Answer 16

ANCA (especially C-ANKA)
FBC- may have reduced Hb
Chest xray 
CT of sinuses
Lung biopsy

Question 17

Which vessels does Kawasaki’s disease mainly affect?

Answer 17

Medium vessels

Especially coronary vessels

Question 18

Why are patients with Kawasaki’s disease more at risk of leucocytosis and thrombocytosis?

Answer 18

Endothelial cells damaged so tunica media exposed which increases risk of coagulation (virchow’s triad)

Question 19

What antibody should be tested for in Kawasaki’s disease?

Answer 19

Anti-endothelial cell antibodies

Question 20

Give some signs and symptoms of Kawasaki’s disease?

Answer 20

CRASH and BURN
Conjunctivitis
Rash
Adenopathy 
Strawberry tongue
Hands and feet redness and oedema 

Burn = fever

Question 21

What is the main cause of death in Kawasaki’s disease?

Answer 21

Myocardial Infarction

Question 22

How is Kawasaki’s disease treated?

Answer 22

IV immunoglobulin

High dose aspirin (at risk of Reyes disease which is a rapidly progressing encephalopathy)

Question 23

What is Takayasu’s arteritis also known as?

Answer 23

Pulselessness disease

Aortic arch syndrome

Question 24

What vessels does Takayasu’s arteritis affect?

Answer 24

Large vessels

Question 25

Give 5 signs/symptoms in Takayasu's arteritis

Answer 25

``` Absent peripheral pulses Hypertension Heart failure Peripheral claudication Neurological symptoms Stroke ```

Question 26

How would Takayasu's be investigated?

Answer 26

Contrast Angiography CT MRI (No autoantibodies associated)

Question 27

How is Takayasu's arteritis treated?

Answer 27

Corticosteroids | Surgical bipass

Question 28

What type of vessels does polyalgia rheumatica effect?

Answer 28

Large vessels

Question 29

What are the characteristic symptoms of polyalgia rheumatica?

Answer 29

Sudden onset pain and stiffness in shoulders and pelvis Normal muscle strength Worse in the morning and improves on exercise ``` Tiredness Fever Weight loss Depression Night sweats Loss of appetite ```

Question 30

What investigations should be done in polyalgia rheumatica?

Answer 30

``` ESR/CRP FBC- normocytic anaemia LFTs - may have raised ALP Temporal artery biopsy CK Muscle biopsy ```

Question 31

How is polyalgia rheumatica treated?

Answer 31

Corticosteroids

Question 32

What are the symptoms of giant cell arteritis?

Answer 32

``` Headache- abrupt onset (over the temples) Scalp tenderness Jaw claudication Sudden loss of vision Malaise Tiredness Fever Thickened temporal arteries (plus symptoms of polymyalgia rheumatica) ```

Question 33

How should giant cell arteritis be investigated?

Answer 33

``` Temporal artery biopsy FBC- normocytic anaemia ESR CRP LFTs ```

Question 34

How is giant cell arteritis managed?

Answer 34

Early treatment Corticosteroids Osteoporosis prophylaxis Methotrexate

Question 35

What is Behcet's disease?

Answer 35

Mainly seen in Turkey, Iran and Japan Unknown cause Systemic vasculitis

Question 36

What are some of the signs and symptoms of Behcet's disease?

Answer 36

``` Oral ulcers Genital ulcers Eye lesions Skin lesions Peripheral arthritis GI symptoms Leg thrombophlebitis ```

Question 37

How is Behcet's investigated?

Answer 37

Pethergy reaction- skin test ESR and CRP raised No specific autoantibodies

Question 38

How is Behcet's disease treated?

Answer 38

``` Corticosteroids Immunosuppressants Ciclosporin Colchicine Anti-TNFs (serious cases) ```