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Flashcards in Vasculitis Deck (60):
1

What is vasculitis?

Inflammation of blood vessels

2

How can vasculitis be classified? (2)

  • Size of vessel
  • ANCA positive or negative

3

What are the two major categories of large vessel vasculitis?

  1. Giant cell arteritis
  2. Takayasu arteritis

4

Which arteries are most common for giant cell arteritis to affect?

  • Temporal artery (most common)
  • Opthalmic artery
  • Facial artery

5

The endothelium is generally directly attacked with molecular mimicry in which two classes of vasculitis?

  • Large vessel
  • Medium vessel

6

The endothelium is generally indirectly attacked in which class of vasculitis?

Small vessel

(N.b Henloch-Scholein purpura is an exeption to this rule)

7

What happens to the vessel walls due to the inflammation is vasculitis?

  • Inflammatory processes cause tissue factor and collagen to be exposed (endothelial cells die)
  • Thrombosis occurs
  • Vessel walls weak leading to aneurysm
  • Fibrosis due to fibrin deposition occurs as part of the healing process
  • Both thrombosis and fibrosis narrow the vessel lumen reducing blood flow

8

Ultimately, vasculitis leads to less ______ _____

Bloof flow

9

Why does jaw claudication occur with giant cell arteritis?

The facial artery is affected and less blood flow to the face occurs

10

Giant cell arteritis is most common in what age group and sex?

Women > 50

11

What is found in the elastic lamina in a patient with giant cell arteritis?

Granulomas or "giant cells"

12

When taking a biopsy for giant cell arteritis, why is a negative result not able to rule out the condition?

Giant cell arteritis is segmental

13

Takayasu arteritis is most common in which age group and sex?

Asian women < 40

14

Takayasu arteritis generally affects which arteries?

Those off of the aortic arch

15

In patients with Takayasu arteritis, what is found in the internal elastic lamina?

Granulomas or "giant cells"

16

What are some common medium vessel arteritides?

  1. Kawasaki disease
  2. Polyarteritis nodosa

17

Kawasaki disease generally affects people of what age?

< 5

(boys more than girls)

18

What are the symptoms of Kawasaki disease?

Conjuctivitis (sparing the limbus)

Rash over body

Adenopathy (large or swollen lymph nodes)

Strawberry tongue

Hands and feet swelling

Fever

(Patients crash and burn)

19

Kawasaki disease is usually _____ __________

Self limiting

20

How can Kawasaki disease be treated?

IV Ig

Aspirin (monitor very closely for Reye syndrome)

21

What is the pathogenesis behind polyarteritis nodosa?

Immune cells directly attack the endothelial cells

This is an example of molecular mimicry as the antibodies produced are designed to takle hepatitis B

22

Polyarteritis nodosa causes ___________ necrosis

Transmural

23

Fibrinoid necrosis occurs with polyarteritis nodosa which leaves the vessels prone to what?

Aneurysm

24

Upon angiogram, polyarteritis nodosa has a very distinctive appearance. What is this appearance?

"String of beads"

25

In polyarteritis nodosa what happens when renal arteries are affected?

Hypertension

(low glomerular pressure leads to fluid retention and increased blood pressure)

26

What can occur when the mesenteric arteries are affected in polyarteritis nodosa?

  • Abdominal pain
  • GI bleed

27

What is Buerger's disease?

A medium vessle vasculitis affecting vessels in the fingers and toes

Blood clots occur as a result leading to ulceration, necrosis and auto-amputation of the fingers and toes

28

What is a major risk factor and potential trigger for Buerger's disease

Use of tobacco

29

What is ANCA?

Anti-neutrophil cytoplasmic antibody

30

Wegener's granulomatosis (Granulomatosis with polyangiitis) involves which type of ANCA?

cANCA

(cytoplasmic ANCA)

31

In Wegener's granulomatosis, cANCA binds to what?

Proteinase 3 within the neutrophilic membrane

32

When pANCA or cANCA binds to neutrophils, why does the endothelium end up damaged?

O2 free radicals are released from neutrophils causing indirect damage

33

Name some small vessel vasculitides?

  • Wegener's granulomatosis
  • Microscopic polyangiitis
  • Churg-Strauss syndrome
  • Henloch-Schonlein purpura
  • Renal limited vasculitis

34

Wegener's granulomatosis is more common in which sex?

Men

35

Which areas of the body does Wegener's granulomatosis commonly affect?

Nasopharynx (painful sinusitis, bloody mucous from ulcers and saddle nose deformity)

Lungs (difficulty breathign due to ulcer formation and bloody cough)

Kidneys

36

Saddle nose deformity is associated with which vasculitis?

Wegener's granulomatosis

37

Once cured, relapse of Wegener's granulomatosis is what?

Common

38

How is microscopic polyangiitis different from Wegener's granulomatosis?

  1. Does not affect nasopharynx (only kidneys and lungs)
  2. No granulomas can be found in vessle walls
  3. pANCA vs cANCA

39

What is pANCA?

Perinuclear ANCA

40

After microscopic polyangiitis is cured or overcome, relapse is what?

Common

41

Churg-Strauss syndrome is commonly mistaken for what?

Asthma or allergies

42

Which immune cell type is particularly elevated in Churg-Strauss syndrome?

Eosinophils

43

Which areas of the body does Churg-Strauss syndrome affect?

  • Sinuses
  • Lungs
  • Kidneys
  • GI tract
  • Skin
  • Nerves
  • Heart

44

Are granulomas associated with Churg-Strauss syndrome?

Yes

45

What makes Henloch-Schonlein purpura different from other small vessel vasculitides?

There are no ANCAs involved

46

What causes Henloch-Schonlein purpura?

IgA targets endothelial cells via molecular mimicry

47

The purpura seen in Henloch-Schonlein purpura is distinctive, but why?

It is palpable

This is due to the fibrosis occurng in the affected vessels

48

Henloch-Schonlein purpura can affect two other major body systems besides the skin, what are these?

GI (abdominal pain)

Renal (haematuria, IgA nephropathy)

49

Henloch-Schonlein purpura can resolve on its own.

True or false?

True

50

Large vessel vasculitis can lead to reduced _______ and carotid ________

Pulses

Bruit

51

What is the treatment for large vessle vasculitis?

40-60mg of prednisolone which granually reduces

52

What is the most common complication of microscopic polyangiitis?

Glomerulonephritis

53

Most cases of ANCA +ve vasculitis require what treatment?

IV steroids

Cyclophosphamide

54

Henloch-Schonlein purpura commonly affects children and a history involving what is common?

An upper respiratory tract infection predating symptom onset by a few weeks

55

Henloch-Schonlein purpura has what symptoms commonly?

  • Purpuric rash over buttocks and lower limbs
  • Abdominal pain
  • Vomiting
  • Joint pain

56

Henloch-Schonlein purpura requires what treatment?

It is a self limiting condition which does nto require much specific treatment

It will settle over a period of weeks to months

57

Temporal arteritis has a common association with what?

Polymyalgia rheumatica

58

cANCA and anti-PR3 are asscoiated with what?

Wegener's granulomatosis

59

pANCA and anti-MPO are associated with what?

Microscopic polyangiitis

60

Anti-MPO or anti-PR3 are asscoiated with what?

Churg-Strauss syndrome

(Eosinophilic granulomatosis with polyangiitis)

Decks in The Musculoskeletal System Class (58):