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Flashcards in Vasculitis Deck (35):
1

What is vasculitis?

Inflammation of blood vessels

2

What vessel changes would be expected in vasculitis?

  1. Vessel wall thickening
  2. Vessel wall stenosis
  3. Occlusion of vessels with subsequent infarction

3

What does the term "large vessel vasculitis" refer to?

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

4

What are the two major categories of large vessel vasculitis?

  1. Giant cell arteritis
  2. Takayasu arteritis

5

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

  • Giant cell arteritis > 50 years
  • Takayasu arteritis < 50 years

6

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

East Asian countries

Females (young 20-30)

7

Giant cell arteritis typically affects which arteries?

Temporal arteries

8

Early features of large vessel vasculitis include which things?

  • Low-grade fever
  • Malaise
  • Night sweats
  • Weight loss
  • Arthralgia
  • Fatigue
  • Claudicant symptoms

9

If untreated, what can occur with large vessel vasculitis?

  1. Vascular stenosis
  2. Aneurysms
  3. Reduced pulses and bruit

10

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

MR angiography

11

What is the rough dosage of steroids for

a) Large vessel vasculitis

b) Giant cell arteritis with ocular involvement

a) 40mg

b) 60mg

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

12

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

Methotrexate or azathioprine

13

Which small vessel vasculitides are ANCA associated?

  1. Granulomatosis with polyangiitis
  2. Microscopic polyangitiis
  3. Eosinophilic granulomatosis with polyangiitis
  4. Renal limited vasculitis

14

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

Microscopic polyangiitis

15

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

Eosinophilic granulomatosis with polyangiitis

16

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

Henoch-Schnlein purpura

17

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

Cryoglobulinaemia

18

Describe what granulomatosis with polyangiitis is

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

Necrotising glomerlulonephritis is common

19

Describe what microscopic polyangiitis is

Necrotising vasculitis affecting small vessels

Both necrotising glomerulonephritis and pulmonary capillaritis are common

20

Describe what renal limitied vasculitis (Pauci-immune) is 

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

Describe what eosinophilic granulomatosis with polyangiitis is

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

Which types of symptoms are common in granulomatosis with polyangiitis?

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

GPA is associated with ______ and ____

cANCA and PR3 (proteinase 3)

24

How is EGPA characterised?

Late onset asthma, rhinitis and raised peripheral blood eosinophil count

25

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

Neurological symptoms

Mononeuritis multiplex

26

EGPA is associated with ____ and ___

pANCA and MPO (myeloperoxidase)

27

What is the most important complication of microscopic polyangiitis?

Glomerulonephritis

(up to 90% of patients experience)

28

Which investigations are appropriate for vasculitis?

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

29

How are most cases of ANCA associated vasculitis treated?

  1. IV steroids
  2. Cyclophosphamide

30

What is Henoch-Schonlein purpura?

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

31

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

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

32

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

Children

33

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

Upper respiratory tract infection

34

Common symptoms of Henoch-Scholein purpura include what?

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

35

What is the treatment for Henoch-Scholein purpura?

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

It will usually settle over the course of weeks and months

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