Vasculitis Flashcards

1
Q

What is vasculitis?

A

Inflammation of blood vessels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What vessel changes would be expected in vasculitis?

A
  1. Vessel wall thickening
  2. Vessel wall stenosis
  3. Occlusion of vessels with subsequent infarction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What does the term “large vessel vasculitis” refer to?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the two major categories of large vessel vasculitis?

A
  1. Giant cell arteritis
  2. Takayasu arteritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How does the age of onset differ in patients with giant cell arteritis vs Takayasu arteritis?

A
  • Giant cell arteritis > 50 years
  • Takayasu arteritis < 50 years
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Where is Takayasu arterits most common in the world and which gender does it tend to affect?

A

East Asian countries

Females (young 20-30)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Giant cell arteritis typically affects which arteries?

A

Temporal arteries

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Early features of large vessel vasculitis include which things?

A
  • Low-grade fever
  • Malaise
  • Night sweats
  • Weight loss
  • Arthralgia
  • Fatigue
  • Claudicant symptoms
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

If untreated, what can occur with large vessel vasculitis?

A
  1. Vascular stenosis
  2. Aneurysms
  3. Reduced pulses and bruit
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Which imaging technique can detect thickened vessel walls and stenosis in large vessel vasculitis?

A

MR angiography

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the rough dosage of steroids for

a) Large vessel vasculitis
b) Giant cell arteritis with ocular involvement

A

a) 40mg
b) 60mg

(1mg/Kg up to a max of 80mg)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

As well as steroids, what other treatments can be added for large vessel vasculitis?

A

Methotrexate or azathioprine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Which small vessel vasculitides are ANCA associated?

A
  1. Granulomatosis with polyangiitis
  2. Microscopic polyangitiis
  3. Eosinophilic granulomatosis with polyangiitis
  4. Renal limited vasculitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Which small vessel vasculitis is associated with ANCA, but not with granuloma?

A

Microscopic polyangiitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Which small vessel vasculitis is ANCA associated, has granulomas present and is also associated with asthma and eosinophilia?

A

Eosinophilic granulomatosis with polyangiitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Which non-ANCA associated small vessel vasculitis involves and IgA dominant immune deposit?

A

Henoch-Schnlein purpura

17
Q

Which non-ANCA associated small vessel vasculitis has high levels of cryoglobulin?

A

Cryoglobulinaemia

18
Q

Describe what granulomatosis with polyangiitis is

A

Granulomatous inflammation involving the respiratory tract combined with necrotising vasculitis of small and medium sized vessels.

Necrotising glomerlulonephritis is common

19
Q

Describe what microscopic polyangiitis is

A

Necrotising vasculitis affecting small vessels

Both necrotising glomerulonephritis and pulmonary capillaritis are common

20
Q

Describe what renal limitied vasculitis (Pauci-immune) is

A

There is Pauci-immune vasculitis (little evidence for hypersensitivity when staining for IgG) and no apparent extra-renal features on presentation.

There is only necrotising glomerulonephritis present

21
Q

Describe what eosinophilic granulomatosis with polyangiitis is

A

Granulomatous inflammation in the respiratory tract

Necrotising vasculitis affecting small and medium sized vessels is present

The condition is associated with eosinophilia and asthma

22
Q

Which types of symptoms are common in granulomatosis with polyangiitis?

A

ENT symptoms

  • Nose bleeds
  • Deafness
  • Recurrent sinusitis
  • Nasal crusting
  • Saddle nose damage

There may also be haemoptysis and cavitating lesions on CXR

Rhinitis is often the first sign

23
Q

GPA is associated with ______ and ____

A

cANCA and PR3 (proteinase 3)

24
Q

How is EGPA characterised?

A

Late onset asthma, rhinitis and raised peripheral blood eosinophil count

25
Q

Whcih other symptoms besides the characteristic symptoms of EGPA are also common?

A

Neurological symptoms

Mononeuritis multiplex

26
Q

EGPA is associated with ____ and ___

A

pANCA and MPO (myeloperoxidase)

27
Q

What is the most important complication of microscopic polyangiitis?

A

Glomerulonephritis

(up to 90% of patients experience)

28
Q

Which investigations are appropriate for vasculitis?

A
  • ESR, PV and CRP (raised)
  • FBC (anaemia is common)
  • U+E (renal involvement)
  • ANCA
  • Urinalysis (renal vasculitis)
  • CXR
  • Angiogram
  • Biopsy of affected area
29
Q

How are most cases of ANCA associated vasculitis treated?

A
  1. IV steroids
  2. Cyclophosphamide
30
Q

What is Henoch-Schonlein purpura?

A

An acute IgA-mediated disorder characterised by a generalised multi-system small vessel vasculitis

31
Q

Which areas of the body does Henoch-Scholein purpura typically affect?

A
  • GI tract
  • Kidneys
  • Joints
  • Lungs (More rare)
  • CNS (more rare)
32
Q

Henoch-Scholein purpura typically affect what age group of people?

A

Children

33
Q

Henoch-Scholein purpura is usually preceeded by a history of what?

A

Upper respiratory tract infection

34
Q

Common symptoms of Henoch-Scholein purpura include what?

A
  1. Purpuric rash over buttocks and lower limbs
  2. Abdominal pain
  3. Vomiting
  4. Joint pain
35
Q

What is the treatment for Henoch-Scholein purpura?

A

It is genrally a self-limiting condition which doesn’t require specific treatment

It will usually settle over the course of weeks and months