Vasculitis Flashcards

1
Q

What is vasculitis?

A

Inflammation of the blood vessel wall

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2
Q

What are the three layers of the arterial wall?

A

Endothelial intima (inner most)
Smooth Muscle media
Connective tissue adventitia

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3
Q

What is the etiology of vasculitis?

A

Usually unknown, most cases are not infectious

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4
Q

What are the clinical features of vasculitis?

A
  1. Nonspecific symptoms of inflammation (e.g. fever, fatigue, weight loss, and myalgias)
  2. Symptoms of organ ischemia (due to luminal narrowing (from fibroblasts) or thrombosis (endothelial cells taken off and under part exposed - attracts platelets) of the inflamed vessels
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5
Q

What does large vessel vasculitis involve?

A

Aorta and its major branches

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6
Q

What does medium vessel vasculitis involve?

A

Muscular arteries that supply organs

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7
Q

What does small vessel vasculitis involve?

A

Arterioles, capillaries and venules

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8
Q

What groups is vasculitis divided into?

A
  • Large-vessel
  • Medium-vessel
  • Small-vessel
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9
Q

What are two types of Large Vessel vasculitis?

A
  • Temporal (Giant Cell) Arteritis

- Takaysu Arteritis

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10
Q

What is Temporal (Giant Cell) Arteritis?

A

Granulomatous vasculitis that classically involves branches of the carotid artery

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11
Q

What is the usual population associated with Temporal (Giant Cell) Arteritis?

A

Females

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12
Q

What is the most common form of vasculitis in older adults?

A

Older adults >50 yrs

Temporal (Giant Cell) Arteritis

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13
Q

What is the typical presentation of Temporal (Giant Cell) Arteritis?

A
  • Headache (temporal artery involvement)
  • Visual disturbances (ophthalmic artery involvement)
  • Jaw claudication
  • Flu-like symptoms
  • Joint and muscle pain (polymyalgia rheumatica)
  • ESR is elevated (often >100)
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14
Q

What does biopsy show in Temporal (Giant Cell) Arteritis?

A

Inflamed vessel wall (vasculitis) with giant cells and intimal fibrosis.

  • Requires biopsy of a long segment of vessel
  • A negative biopsy does not exclude disease
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15
Q

What are the features of lesions in Temporal (Giant Cell) Arteritis?

A

Lesions are segmental

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16
Q

How do you treat Temporal (Giant Cell) Arteritis?

A

Corticosteroids

-High risk of blindness without treatment so you need to treat ASAP

17
Q

What is Takayasu Arteritis?

A

Granulomatous vasculitis that classically involves the aortic arch at branch points.

18
Q

What is the usual population that presents with Takayasu Arteritis?

A

Adults

19
Q

What symptoms does Takayasu Arteritis usually present with?

A

-Neurological and visual symptoms with a weak or absent pulse in the upper extremity (‘pulseless disease’)

20
Q

What do labs show in Takayasu Arteritis?

A

Elevated ESR

21
Q

What is the treatment of Takayasu Arteritis?

A

Corticosteroids

22
Q

What are the three types of Medium Vessel Vasculitis?

A
  1. Polyarteritis Nodosa
  2. Kawasaki Disease
  3. Buerger Disease
23
Q

What is Polyarteritis Nodosa?

A

Necrotizing vasculitis involving multiple organs

  • Lungs are spared
  • Involves “many arteries”
24
Q

What symptoms does Polyarteritis Nodosa present with?

A

Usually presents in young adults as:

  • Hypertension (renal artery involvement)
  • Abdominal pain with melena (black ‘tarry’ feces) (mesenteric artery involvement)
  • Neurologic disturbances
  • Skin lesions
25
Q

What does Medium-Vessel Vasculitis involve?

A

Muscular arteries that supply organs (ex: renal artery)

26
Q

What lab is Polyarteritis Nodosa associated with?

A

Serum HBsAg

-Hep B surface antigens found in these patients

27
Q

What types of lesions do you see with Polyarteritis Nodosa?

A
  • Lesions of varying stages are present
  • Early lesion consists of transmural inflammation with fibrinoid necrosis (highlighter pink)
  • This fibrinoid necrosis eventually heals with fibrosis, producing a ‘string-of-pearls’ appearance on imaging
28
Q

What do you see upon imaging of Polyarteritis Nodosa?

A

“String-of-pearls” appearance on imaging

-Caused by aneurysms

29
Q

How do you treat Polyarteritis Nodosa?

A

-Corticosteroid
and
-Cyclophosphamide

30
Q

What can happen if you do not treat Polyarteritis Nodosa?

A

It can become fatal.