Vasculitides Flashcards

1
Q

Name the Large-Vessel Vasculitides

A
Giant Cell (Temporal) Arteritis
Takayasu Arteritis
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2
Q

Name the Medium-Vessel Vasculitides

A
Polyarteritis Nodosa
Kawasaki Disease
Burger Disease (Thromboangiitis Obliterans)
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3
Q

Name the Small-Vessel Vasculitides

A

Granulomatosis with Polyangiitis (Wegener)
Microscopic Polyangiitis
Eosinophilic Granulomatosis with polyangiitis (Churg-Strauss)
Henoch-Schonlein Purpura

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4
Q

Medium-vessel vasculitis involves what type of arteries? HY

A

muscular arteries that supply organs

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5
Q

Etiology of most vasculitis is what?

A

unknown and not infectious

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6
Q

What arteries does Giant Cell Arteritis usually involve?

A

branches of the carotid artery

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7
Q

what type of vasculitis is Giant Cell Arteritis?

A

Granulomatous (always with giant cell)

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8
Q

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

A

Giant Cell Arteritis

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9
Q

Giant Cell Arteritis usually effects what population?

A

usually affects older females

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10
Q

What is polymyalgia rheumatica and what vasculitis is associated with it?

A

flu-like symptoms with joint and muscle pain

Giant Cell Arteritis

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11
Q

What are the issues that arise from the arteries involved in giant cell arteritis?

A

headache - temporal
blindness - ophthalmic
jaw claudication

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12
Q

What blood test is elevated in Giant Cell Arteritis?

A

ESR > 100

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13
Q

Upon biopsy what do you see in giant cell arteritis?

A

inflamed vessel wall with giant cells and intimal fibrosis (focal granulomatous inflammation)

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14
Q

How do you treat Giant Cell Arteritis?

A

corticosteroids

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15
Q

Complication from not treating Giant Cell Arteritis? HY

A

high risk of blindness

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16
Q

What type of vasculitis is Takayasu and what arteries does is classically involve?

A

granulomatous vasculitis classically affecting aortic arch at branch points

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17
Q

Who gets Takayasu arteritis?

A

young Asian females

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18
Q

What arteritis is known as pulseless disease and what does that present with?

A

Takayasu

weak or absent pulse in upper extremity

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19
Q

What are some signs and symptoms of Takayasu?

A

flu-like symptoms with neurological and ocular disturbances along with a weak or absent upper extremity pulse

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20
Q

What blood test is elevated in Takayasu?

A

ESR

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21
Q

What is the treatment for Takayasu?

A

corticosteroids

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22
Q

Polyarteritis Nodosa gets signs and symptoms in every organ except what?

A

lungs

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23
Q

What presents in young adults as hypertension, abdominal pain with melena, neurologic disturbances, and skin lesions?

A

Polyarteritis Nodosa

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24
Q

What serotype is positive in Polyarteritis Nodosa? (HY)

A

serum HBsAg (Hep B)

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25
Q

Early vs Late lesions in Polyarteritis Nodosa?

A

early lesions consists of transmural inflammation with fibrinoid necrosis
late lesions heals with fibrosis

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26
Q

What vasculitis has strings-of-pearls appearance and what causes them? HY

A

Polyarteritis Nodosa?

Early lesions become aneurysms between the late fibrotic lesions

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27
Q

What is the prognosis of Polyarteritis Nodosa?

A

fatal if left untreated

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28
Q

What is the treatment for Polyarteritis Nodosa?

A

Corticosteroids and Cyclophosphamide

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29
Q

What is the immune pathogenicity of Polyarteritis Nodosa?

A

immune complex mediated (Type III Hypersensitivity)

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30
Q

Who gets Kawasaki Disease?

A

Asian children

31
Q

What is one way to remember who gets Kawasaki Disease?

A

think of a young asian child riding a kawasaki bike - palms and soles affected

32
Q

CRASH and Burn mnemonic for Kawasaki Disease?

A
Conjunctival injection
Rash (polymorphous -> desquamating)
Adenopathy (cervical)
Strawberry tongue (oral mucositis)
Hand-Foot erythematous rash
fever
33
Q

Kawasaki Disease commonly can affect what arteries with what complications? HY

A

Coronary artery involvement leading to (1) thrombosis with MI and (2) aneurysm with rupture

34
Q

Treatment of Kawasaki Disease?

A

aspirin and IVIG

35
Q

Another name for Beurger Disease

A

Thromboangiitis Obliterans

36
Q

Who gets Thromboangiitis Obliterans (Beurger)?

A

heavy smokers, it is a smokers disease

Males

37
Q

What is often present in Thromboangiitis Obliterans (Beurger)? HY

A

Raynaud Phenomenon

38
Q

What type of vasculitis is Thromboangiitis Obliterans (Beurger)?

A

necrotizing vasculitis of digits

39
Q

How does Thromboangiitis Obliterans (Beurger) present?

A

gangrene, ulceration, and autoamputation of fingers and toes

40
Q

Treatment for Thromboangiitis Obliterans (Beurger)?

A

smoking cessation

41
Q

What is another name for Wegeners?

A

Granulomatosis with polyangiitis

42
Q

What type of vasculitis is Wegeners?

A

necrotizing granulomatous vasculitis

43
Q

Where does Wegeners commonly affect?

A

Nasopharynx, Lungs, and Kidneys

44
Q

What is the We”C”ener’s mnemonic? HY

A

lots of C’s with this disease
C shape distribution of disease
C-ANCA (PR3-ANCA)
Cyclophosphamide and Corticosteroids treatment

45
Q

What vasculitis is classically seen in a middle aged male presenting with sinusitis, nasopharyngeal ulcers, hemoptysis, bilateral nodular lung infiltrates, and hematuria?

A

Granulomatosis with Polyangiitis (Wegeners)

46
Q

Why do Wegeners patients get hematuria? HY

A

rapidly progressive necrotizing glomerulonephritis

47
Q

What blood marker correlates with disease activity in Wegeners?

A

C-ANCA/PR3-ANCA

48
Q

What does biopsy in Wegeners show?

A

large necrotizing granulomas with adjacent necrotizing vasculitis

49
Q

What is the treatment for Wegeners? HY

A

Cyclophosphamide and corticosteroids

50
Q

Prognosis of Granulomatosis with polyangiitis?

A

relapses are common

51
Q

What is PR3-ANCA?

A

anti-proteinase-3

52
Q

What vasculitis is similar in presentation to Wegeners but lacks nasopharyngeal involvement and has no granulomas?

A

Microscopic Polyangiitis

53
Q

What kind of vasculitis is Microscopic Polyangiitis and what organ does it involve?

A

Necrotizing vasculitis involving especially lung, kidney and skin

54
Q

How does Microscopic Polyangiitis present?

A

pauci-immune glomerulonephritis and palpable purpura

55
Q

What blood marker correlates with disease activity in Microscopic Polyangiitis?

A

P-ANCA/MPO-ANCA

56
Q

How do you differentiate between Wegeners and Microscopic Polyangiitis?

A

Microscopic Polyangiitis has no nasopharyngeal involvement and no granulomas

57
Q

How do you treat Microscopic Polyangiitis?

A

corticosteroids and cyclophosphamide

58
Q

What kind of vasculitis is Churg-Strauss and what organs are involved?

A

Granulomatous, Necrotizing vasculitis with eosinophilia involving especially lungs and heart

59
Q

What vasculitis presents with asthma?

A

Churg-Strauss (Eosinophilic Granulomatosis with Polyangiitis)

60
Q

What vasculitis presents with peripheral neuropathy leading to wrist and foot drop?

A

Churg-Strauss (Eosinophilic Granulomatosis with Polyangiitis)

61
Q

What blood marker correlates with disease activity in Churg-Strauss?

A

P-ANCA/MPO-ANCA

62
Q

How do you differentiate between Microscopic Polyangiitis and Churg-Strauss?

A

both have MPO/P-ANCA
Microscopic Polyangiitis has no asthma and no granulomas
Churg Strauss has granulomas, eosinophilia, and asthma

63
Q

What type of kidney involvement is there in Churg-Strauss?

A

pauci-immune glomerulonephritis

64
Q

What does pauci-immune mean?

A

vasculitis that is associated with minimal evidence of hypersensitivity upon immunofluorescence.

65
Q

What is the most common vasculitis in children? HY

A

Henoch Schonlein Purpura

66
Q

What does Henoch Schonlein Purpura occurs following what?

A

upper respiratory tract infection

67
Q

What is Henoch Schonlein Purpura secondary to? HY

A

IgA immune complex deposition

68
Q

Pathogenesis of Henoch Schonlein Purpura HY

A

Upper respiratory infection leads to high IgA due to mucosal involvement –> accumulation of IgA –> IgA complex deposition –> vasculitis

69
Q

What is a major complication of igA deposition in Henoch Schonlein Purpura? HY

A

hematuria due to IgA nephropathy (Berger Disease)

70
Q

What sign occurs in Henoch Schonlein Purpura due to inflammation? HY

A

PALPABLE purpura on buttocks and legs

71
Q

Classic triad in Henoch Schonlein Purpura?

A

palpable purpura on buttocks/legs
Arthralgias
abdominal pain

72
Q

Treatment for Henoch Schonlein Purpura?

A

self limited disease but may recur, treat with steroids if severe

73
Q

What vasculitis presents with melena?

A

Polyarteritis Nodosa