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Path U2 > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis?

A

Inflammation of the blood vessel wall

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2
Q

What is the arterial wall comprised of?

A

three layers: endothelial intima, smooth muscle media, and connective tissue adventitia

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3
Q

What is the etiology for vasculitis?

A

usually unknown; most cases are not infectious.

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4
Q

What do the clinical features for vasculitis include?

A

1) Nonspecific symptoms of inflammation (e.g., fever, fatigue, weight loss, and myalgias) 2) Symptoms of organ ischemia—due to luminal narrowing or thrombosis of the inflamed vessels

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5
Q

Why are there symptoms of organ ischemia in vasculitis?

A

Its due to luminal narrowing or thrombosis of the inflamed vessels

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6
Q

How is vasculitis divided?

A

into large-, medium-, and small-vessel vasculitides

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7
Q

What does large-vessel vasculitis involve?

A

the aorta and its major branches.

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8
Q

What does medium-vessel vasculitis involve?

A

muscular arteries that supply organs.

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9
Q

What does small-vessel vasculitis involve?

A

arterioles, capillaries, and venules.

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10
Q

What are the large vessel vasculitis?

A

Temporal (Giant Cell) Arteritis and Takayasu Arteritis

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11
Q

What is temporal (Giant Cell) Arteritis?

A

Granulomatous vasculitis that classically involves branches of the carotid artery

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12
Q

What is the most common form of vasculitis in older adults (> 50 years)?

A

temporal (Giant Cell) Arteritis and usually affects females

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13
Q

How does temporal (Giant Cell) Arteritis present?

A

as headache (temporal artery involvement), visual disturbances (ophthalmic artery involvement), and jaw claudication. Flu-like symptoms with joint and muscle pain (polymyalgia rheumatica) are often present. ESR is elevated.

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14
Q

What does biopsy of temporal (Giant Cell) Arteritis reveal?

A

inflamed vessel wall with giant cells and intima fibrosis

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15
Q

How do the lesions appear?

A

They are segmental;

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16
Q

What does diagnosis of temporal (Giant Cell) Arteritis require?

A

biopsy of a long segment of vessel, and a negative biopsy does not exclude disease.

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17
Q

What is the treatment for temporal (Giant Cell) Arteritis?

A

it is corticosteroids; high risk of blindness without treatment

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18
Q

What is Takayasu Arteritis?

A

Granulomatous vasculitis that classically involves the aortic arch at branch points

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19
Q

Takayasu Arteritis presents in?

A

adults < 50 years old (classically, young Asian females) as visual and neurologic symptoms with a weak or absent pulse in the upper extremity (‘pulseless disease’). ESR is elevated.

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20
Q

What is the treatment for Takayasu Arteritis?

A

it is corticosteroids

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21
Q

What are the medium vessel vasculitis?

A

Polyarteritis Nodosa, Kawasaki Disease, Buerger Disease

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22
Q

What is Polyarteritis Nodosa?

A

Necrotizing vasculitis involving multiple organs; lungs are spared.

23
Q

How does Polyarteritis Nodosa classically present?

A

in young adults as hypertension (renal artery involvement), abdominal pain with melena (mesenteric artery involvement), neurologic disturbances, and skin lesions.

24
Q

What is Polyarteritis Nodosa associated with?

A

serum HBsAg

25
Q

In Polyarteritis Nodosa what types of lesions are present?

A

Lesions of varying stages are present.

26
Q

What does the early lesion of Polyarteritis Nodosa consist of?

A

transmural inflammation with fibrinoid necrosis that eventually heals with fibrosis,

27
Q

How does the early lesion of Polyarteritis Nodosa appear on imaging?

A

It produces a string-of-pearls appearance on imaging

28
Q

What is the treatment for Polyarteritis Nodosa?

A

corticosteroids and cyclophosphamide; fatal if not treated

29
Q

Who does Kawasaki Disease classically affect?

A

Asian children < 4 years old

30
Q

How does Kawasaki disease present?

A

with nonspecific signs including fever, conjunctivitis, erythematous rash of palms and soles, and enlarged cervicai lymph nodes

31
Q

In Kawasaki disease involvement of what is common and what does this usually lead to?

A

Coronary artery involvement is common and leads to risk for (1) thrombosis with myocardial infarction and (2) aneurysm with rupture.

32
Q

What is the treatment for Kawasaki disease?

A

It is aspirin and IVIG; disease is self-limited.

33
Q

What is Buerger Disease?

A

Necrotizing vasculitis involving digits

34
Q

How does Buerger Disease?

A

Presents with ulceration, gangrene, and autoamputation of fingers and toes

35
Q

In Buerger Disease, what is often present?

A

Raynaud phenomenon

36
Q

Buerger Disease is highly associated with what patient behavior?

A

heavy smoking; treatment is smoking cessation.

37
Q

What are the small vessel vasculitis?

A

Wegener Granulomatous, microscopic polyangiitis, Churg-Strauss Syndrome, Henoch-Schönlein Purpura

38
Q

What is Wegener Granulomatosis?

A

Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys

39
Q

What is the classic presentation for Wegener Granulomatosis?

A

the classic presentation is a middle-aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis.

40
Q

In Wegener Granulomatosis how can you gauge disease activity?

A

Serum c-ANCA levels correlate with disease activity,

41
Q

What does Wegener Granulomatosis Biopsy reveal?

A

large necrotizing granulomas with adjacent necrotizing vasculitis

42
Q

What is the treatment for Wegener Granulomatosis?

A

it is cyclophosphamide and steroids; relapses are common,

43
Q

What is microscopic polyangiitis?

A

Necrotizing vasculitis involving multiple organs, especially lung and kidney

44
Q

What is the presentation for microscopic polyangiitis?

A

It is similar to Wegener granulomatosis, but nasopharyngeal involvement and granulomas are absent.

45
Q

How can you gauge microscopic polyangiitis disease activity?

A

Serum p-ANCA levels correlate with disease activity

46
Q

What is the treatment for microscopic polyangiitis?

A

It is corticosteroids and cyclophosphamide; relapses are common.

47
Q

What is Churg-Strauss Syndrome?

A

Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially lungs and heart

48
Q

In Churg-Strauss Syndrome what else are also present in addition to the necrotizing granulomatous inflammation?

A

Asthma and peripheral eosinophilia are often present.

49
Q

How could you gauge Churg-Strauss Syndrome?

A

Serum p-ANCA levels correlate with disease activity.

50
Q

What is Henoch-Schönlein Purpura?

A

Vasculitis due to IgA immune complex deposition

51
Q

What is the most common vasculitis in children?

A

Henoch-Schönlein Purpura

52
Q

How does Henoch-Schönlein Purpura present?

A

with palpable purpura on buttocks and legs, CI pain and bleeding, and hematuria (IgA nephropathy);

53
Q

Henoch-Schönlein Purpura usually occurs following what?

A

an upper respiratory tract infection

54
Q

How is Henoch-Schönlein Purpura treated?

A

Disease is self-limited, but may recur; treated with steroids, if severe

Path U2 (25 decks)

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