Vasculitic Syndromes Flashcards

1
Q

Giant cell arteritis- epidemiologies

A

females of north european descent, >50

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2
Q

Giant cell arteritis- Sx

A

it’s in the temporal artery so headaches, jaw pain, blurry/double vision

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3
Q

Giant cell arteritis- Dx

A

bilateral temporal artery biopsy, increase in ESR, multinucleated giant cells

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4
Q

Giant cell arteritis- Tx

A

prednisone

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5
Q

Takayasu arteritis- what is it

A

also known as aortic arch syndrome or pulseless disease. Vasculitis causes an inflammation which damages the aorta and the its main branches leading to arterial stenosis or aneurysms.

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6
Q

Takayasu arteritis- patient population

A

females between 15 and 40 years old in Asia, Latin America, and Eastern Europe

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7
Q

Takayasu arteritis- Sx

A

abscence of peripheral pulses, ischemia to head, fever, vascular bruits

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8
Q

Takayasu arteritis- Dx

A

increase in ESR and aortic arch thickening

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9
Q

Takayasu arteritis- Tx

A

Corticosteroids

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10
Q

What is pylyarteritis nodosa (PAN)?

A

vasculitis of medium-sized vessels

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11
Q

Is PAN involved with glomerulonephritis?

A

No

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12
Q

What infection is PAN assocated with?

A

Hep B and C

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13
Q

PAN- Dx

A

increase in BUN and Hep B antigen

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14
Q

What is Microscopic polyangitis (MPA)?

A

vasculitis of small-sized vessels

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15
Q

Is MPA involved with glomerulonephritis?

A

Yes (+lung involvement)

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16
Q

MPA- Dx

A

Increase in P-ANCA

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17
Q

What are the diseases associated with secondary polyarteritis?

A

hepatitis C infection, rheumatoid arthritis, Sjogren syndrome, mixed cryoglobulinemia, hairy cell leukemia, myelodysplastic syndrome, and other hematologic malignancies

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18
Q

What is the confirmatory test used to demonstrate vasculitis?

A

Angiography or biopsy of involved tissue showing vasculitis

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19
Q

What are the agents used in the treatment of vasculitis?

A

Early diagnosis and corticosteroid therapy. Cytotoxic or antimetabolite drugs such as cyclophosphamide, methotrexate, and azathioprine are used in combination with corticosteroids

20
Q

Churg Strauss vasculitis- why it’s different than PAN

A

The presence of granulomas as well as the abundance of eosinophils distinguishes this disease from PAN

21
Q

Churg Strauss vasculitis- population

A

males with a median age of 38

22
Q

Churg Strauss vasculitis- Sx

A

1) a history of, or current symptoms of, asthma, 2) peripheral eosinophilia (>1.5 x 10^9 eosinophils/L), 3) systemic vasculitis of at least 2 extra-pulmonary organs.

23
Q

Churg Strauss vasculitis- Dx

A

Granulomatous, necrotizing vasculitis w/ eosinophilia. Elevated IgE & ↑ P-ANCA

24
Q

Churg Strauss vasculitis- Tx

A

Corticosteriod w/ or w/o cytotoxic agents

25
Q

Buerger- population

A

young smokers

26
Q

Buerger disease- Sx

A

ischemic injury to fingers and toes, can lead to amputation

27
Q

Buerger disease- Tx

A

STOP SMOKING YOU IDIOT

28
Q

Primary Angitis of CNS- cause

A

a form of vasculitis where the body attacks the arteries of the brain and spinal cord. It has a chronic fluctuating and progressive course

29
Q

Primary Angitis of CNS- Sx

A

headache, maybe some mild CNS, rarely coma or stroke

30
Q

Primary Angitis of CNS- Dx

A

Cerebral angiography and biopsy of CNS tissues, including the leptomeninges

31
Q

Primary Angitis of CNS- Tx

A

Short, benign course: use corticosteroids and calcium-channel blockers to prevent vasospasm

32
Q

Wegener Granulomatosis- 3 signs

A

upper and lower respiratory tract necrotizing granulomatous inflammation and focal segmental necrotizing glomerulonephritis

33
Q

Wegener Granulomatosis- population

A

40-50 year olds. Slight male predominance.

34
Q

Wegener Granulomatosis- Dx

A

positive cytoplasmic (c-) ANCA test

35
Q

Wegener Granulomatosis- Tx

A

Corticosteroids

36
Q

What are the 4 diseases with a + p-ANCA test?

A

Idiopathic crescentic glomerulonephritis
Microscopic polyarteritis nodosa
Churg-Strauss syndrome
Wegener granulomatosis

37
Q

What is the most common cause of isolated cutaneous vasculitis?

A

drugs

38
Q

What are clinical manifestations of isolated cutaneous vasculitis?

A

urticaria, palpable purpura, livedo reticularis, or skin ulceration.

39
Q

Schonlein-Henoch vasculitis- presentation

A

arthritis, gastrointestinal pain, kidney inflammation, and purpura in kids

40
Q

Schonlein-Henoch vasculitis- Dx

A

IgA deposition in vessel walls and normal complement levels.

41
Q

Type I Cryoglobulins- 3 cancers

A

Multiple myeloma, chronic lymphocytic leukemia and Waldenstrom’s macroglobulinemia are all cancers of this type

42
Q

Type I Cryoglobulins- Sx

A

headaches, visual disturbances, nosebleeds, Raynaud phenomenon, and ischemic ulceration

43
Q

Type I Cryoglobulins- Lab findings

A

increased IgM

44
Q

Type II cryoglobulinemia- 3 associated diseases

A

chronic infections (most commonly hepatitis C), autoimmune disorders, and sometimes lymphoma

45
Q

Type II cryoglobulinemia- presentation

A

nonsystemic small vessel vasculitis with palpable purpura, urticaria, and cutaneous ulceration

46
Q

Type II cryoglobulinemia- lab findings

A

↑erythrocyte sedimentation rate, ↑immunoglobulin levels, positive rheumatoid factor