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MSK Patrick Stenhouse > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis?

A

Inflammation of blood vessels

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2
Q

How can vasculitis be classified? (2)

A
  • Size of vessel
  • ANCA positive or negative
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3
Q

What are the two major categories of large vessel vasculitis?

A
  1. Giant cell arteritis
  2. Takayasu arteritis
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4
Q

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

A
  • Temporal artery (most common)
  • Opthalmic artery
  • Facial artery
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5
Q

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

A
  • Large vessel
  • Medium vessel
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6
Q

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

A

Small vessel

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

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7
Q

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

A
  • 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
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8
Q

Ultimately, vasculitis leads to less ______ _____

A

Bloof flow

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9
Q

Why does jaw claudication occur with giant cell arteritis?

A

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

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10
Q

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

A

Women > 50

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11
Q

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

A

Granulomas or “giant cells”

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12
Q

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

A

Giant cell arteritis is segmental

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13
Q

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

A

Asian women < 40

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14
Q

Takayasu arteritis generally affects which arteries?

A

Those off of the aortic arch

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15
Q

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

A

Granulomas or “giant cells”

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16
Q

What are some common medium vessel arteritides?

A
  1. Kawasaki disease
  2. Polyarteritis nodosa
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17
Q

Kawasaki disease generally affects people of what age?

A

< 5

(boys more than girls)

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18
Q

What are the symptoms of Kawasaki disease?

A

Conjuctivitis (sparing the limbus)

Rash over body

Adenopathy (large or swollen lymph nodes)

Strawberry tongue

Hands and feet swelling

Fever

(Patients crash and burn)

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19
Q

Kawasaki disease is usually _____ __________

A

Self limiting

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20
Q

How can Kawasaki disease be treated?

A

IV Ig

Aspirin (monitor very closely for Reye syndrome)

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21
Q

What is the pathogenesis behind polyarteritis nodosa?

A

Immune cells directly attack the endothelial cells

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

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22
Q

Polyarteritis nodosa causes ___________ necrosis

A

Transmural

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23
Q

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

A

Aneurysm

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24
Q

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

A

“String of beads”

25
Q

In polyarteritis nodosa what happens when renal arteries are affected?

A

Hypertension

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

26
Q

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

A
  • Abdominal pain
  • GI bleed
27
Q

What is ANCA?

A

Anti-neutrophil cytoplasmic antibody

28
Q

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

A

cANCA

(cytoplasmic ANCA)

29
Q

In Wegener’s granulomatosis, cANCA binds to what?

A

Proteinase 3 within the neutrophilic membrane

30
Q

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

A

O2 free radicals are released from neutrophils causing indirect damage

31
Q

Name some small vessel vasculitides?

A
  • Wegener’s granulomatosis
  • Microscopic polyangiitis
  • Churg-Strauss syndrome
  • Henloch-Schonlein purpura
  • Renal limited vasculitis
32
Q

Wegener’s granulomatosis is more common in which sex?

A

Men

33
Q

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

A

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

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

Kidneys

34
Q

Saddle nose deformity is associated with which vasculitis?

A

Wegener’s granulomatosis

35
Q

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

A

Common

36
Q

How is microscopic polyangiitis different from Wegener’s granulomatosis?

A
  1. Does not affect nasopharynx (only kidneys and lungs)
  2. No granulomas can be found in vessle walls
  3. pANCA vs cANCA
37
Q

What is pANCA?

A

Perinuclear ANCA

38
Q

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

A

Common

39
Q

Churg-Strauss syndrome is commonly mistaken for what?

A

Asthma or allergies

40
Q

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

A

Eosinophils

41
Q

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

A
  • Sinuses
  • Lungs
  • Kidneys
  • GI tract
  • Skin
  • Nerves
  • Heart
42
Q

Are granulomas associated with Churg-Strauss syndrome?

A

Yes

43
Q

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

A

There are no ANCAs involved

44
Q

What causes Henloch-Schonlein purpura?

A

IgA targets endothelial cells via molecular mimicry

45
Q

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

A

It is palpable

This is due to the fibrosis occurng in the affected vessels

46
Q

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

A

GI (abdominal pain)

Renal (haematuria, IgA nephropathy)

47
Q

Henloch-Schonlein purpura can resolve on its own.

True or false?

A

True

48
Q

Large vessel vasculitis can lead to reduced _______ and carotid ________

A

Pulses

Bruit

49
Q

What is the treatment for large vessle vasculitis?

A

40-60mg of prednisolone which granually reduces

50
Q

What is the most common complication of microscopic polyangiitis?

A

Glomerulonephritis

51
Q

Most cases of ANCA +ve vasculitis require what treatment?

A

IV steroids

Cyclophosphamide

52
Q

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

A

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

53
Q

Henloch-Schonlein purpura has what symptoms commonly?

A
  • Purpuric rash over buttocks and lower limbs
  • Abdominal pain
  • Vomiting
  • Joint pain
54
Q

Henloch-Schonlein purpura requires what treatment?

A

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

It will settle over a period of weeks to months

55
Q

Temporal arteritis has a common association with what?

A

Polymyalgia rheumatica

56
Q

cANCA and anti-PR3 are asscoiated with what?

A

Wegener’s granulomatosis

57
Q

pANCA and anti-MPO are associated with what?

A

Microscopic polyangiitis

58
Q

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

A

Churg-Strauss syndrome

(Eosinophilic granulomatosis with polyangiitis)

MSK Patrick Stenhouse (60 decks)

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