Vasculitis

Question 1

Define vasculitis

Answer 1

Inflammation of blood vessels; resulting in vessel wall thickening, stenosis, occlusion and subsequent ischaemia

Question 2

What is large cell vasculitis?

Answer 2

Primary vasculitis causing chronic granulomatous inflammation of the aorta and its major branches

Question 3

What are the two main categories of large cell vasculitis?

Answer 3

Temporal (giant cell) arteritis and Takayasu arteritis (TA)

Question 4

When does large vessel vasculitis onset?

Answer 4

Giant cell- rarely before 50

TA- rarely after 50

Question 5

Who is affected by TA?

Answer 5

Young women in second and third decades of life- 100x more common in east asia

Question 6

What does GCA affect?

Answer 6

Temporal arteries but may also (or only) affect the large vessels.

Question 7

What are the early features of large vessel vasculitis?

Answer 7

Low-grade fever
Malaise
Night sweats
Weight loss
Arthlagia 
Fatigue

Question 8

What are the later features of large vessel vasculitis?

Answer 8

Claudicant symptoms in upper and lower limbs

If untreated vascular stenosis and aneurysms occur

Result in reduced pulses and bruits

Question 9

What is elevated in large vessel vasculitis?

Answer 9

ESR, PV, CRP

Question 10

What will MR angiography detect in large vessel vasculitis?

Answer 10

Thickened vessel walls and stenosis

Question 11

What is the mainstay of treatment in large vessel vasculitis?

Answer 11

Corticosteroids, start at 40-60mg predisolone and reduce

Steroid sparing agents; methotrexate and azathioprine may be added

Question 12

What are small to medium vessel vasculitis’ grouped into?

Answer 12

ANCA +ve and ANCA -ve

Question 13

What are the ANCA associated vasculitis’

Answer 13

Wegeners granulomatosis

Microscopic polyangitis

Churg-strauss

Question 14

What are the non-ANCA associated vasculitis’

Answer 14

Henoch-schnlein purpura

Cryoglobulinemia

IBD vasculitis

Question 15

What are the presenting features of small to medium vessel vasculitis?

Answer 15

Fever and weight loss

A raised, non blanching purpuric rash

Arthralgia/arthritis

Mononeuritis multiplex

Glomerulonephritis

Lung opacities on x-ray

Question 16

Describe Wegeners/granulomaosis with polyangitis

Answer 16

ENT symptoms common, nose bleeds, deafness recurrent sinusitis, nasal crusting (collapse of nose)

Haemoptysis and cavitating lesions on x-ray

Question 17

What is GPA associated with?

Answer 17

cANCA and PR3 (proteinase-3)

Question 18

What characterises eosinophilic granulomatosis with polyangitis?

Answer 18

Late onset asthma, rhinitis, raised peripheral blood eosinophil count

Mono neuritis multiplex

Question 19

What is the most common complication of microscopic polyangitis?

Answer 19

Glomerulonephritis (occurs in 90%)

Question 20

What investigations are done in microscopic polyangitis?

Answer 20

ESR, PV and CRP (raised)

Anaemia of chronic disease

U+E looking for renal involvement

Anti-neutrophil cytoplasmic antibody (ANCA)

Urinalysis (looking for renal vasculitis)

CXR

Biopsy of an affected area e.g. skin or kidney is often helpful in confirming the diagnosis

Question 21

How is ANCA associated vasculitis managed?

Answer 21

IV steroids and cyclophosphamide

Question 22

Describe Henoch-schonlein pupura

Answer 22

Acute immunoglobulin A mediated disorder

Generalised vasculitis involving small vessels of skin, GI, kidneys, joints, lungs, CNS

Question 23

What are the symptoms of Henoch-schonlein pupura?

Answer 23

Pupuric rash over buttocks and lower limbs, abdominal pain, vomitting, joint pain

Question 24

What is the treatment for henoch-schonlein pupura?

Answer 24

Self-limiting condition
No specific treatment
Settles weeks/months