Vasculitic Syndromes

Question 1

Giant cell arteritis- epidemiologies

Answer 1

females of north european descent, >50

Question 2

Giant cell arteritis- Sx

Answer 2

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

Question 3

Giant cell arteritis- Dx

Answer 3

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

Question 4

Giant cell arteritis- Tx

Answer 4

prednisone

Question 5

Takayasu arteritis- what is it

Answer 5

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.

Question 6

Takayasu arteritis- patient population

Answer 6

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

Question 7

Takayasu arteritis- Sx

Answer 7

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

Question 8

Takayasu arteritis- Dx

Answer 8

increase in ESR and aortic arch thickening

Question 9

Takayasu arteritis- Tx

Answer 9

Corticosteroids

Question 10

What is pylyarteritis nodosa (PAN)?

Answer 10

vasculitis of medium-sized vessels

Question 11

Is PAN involved with glomerulonephritis?

Answer 11

No

Question 12

What infection is PAN assocated with?

Answer 12

Hep B and C

Question 13

PAN- Dx

Answer 13

increase in BUN and Hep B antigen

Question 14

What is Microscopic polyangitis (MPA)?

Answer 14

vasculitis of small-sized vessels

Question 15

Is MPA involved with glomerulonephritis?

Answer 15

Yes (+lung involvement)

Question 16

MPA- Dx

Answer 16

Increase in P-ANCA

Question 17

What are the diseases associated with secondary polyarteritis?

Answer 17

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

Question 18

What is the confirmatory test used to demonstrate vasculitis?

Answer 18

Angiography or biopsy of involved tissue showing vasculitis

Question 19

What are the agents used in the treatment of vasculitis?

Answer 19

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

Question 20

Churg Strauss vasculitis- why it’s different than PAN

Answer 20

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

Question 21

Churg Strauss vasculitis- population

Answer 21

males with a median age of 38

Question 22

Churg Strauss vasculitis- Sx

Answer 22

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.

Question 23

Churg Strauss vasculitis- Dx

Answer 23

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

Question 24

Churg Strauss vasculitis- Tx

Answer 24

Corticosteriod w/ or w/o cytotoxic agents

Question 25

Buerger- population

Answer 25

young smokers

Question 26

Buerger disease- Sx

Answer 26

ischemic injury to fingers and toes, can lead to amputation

Question 27

Buerger disease- Tx

Answer 27

STOP SMOKING YOU IDIOT

Question 28

Primary Angitis of CNS- cause

Answer 28

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

Question 29

Primary Angitis of CNS- Sx

Answer 29

headache, maybe some mild CNS, rarely coma or stroke

Question 30

Primary Angitis of CNS- Dx

Answer 30

Cerebral angiography and biopsy of CNS tissues, including the leptomeninges

Question 31

Primary Angitis of CNS- Tx

Answer 31

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

Question 32

Wegener Granulomatosis- 3 signs

Answer 32

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

Question 33

Wegener Granulomatosis- population

Answer 33

40-50 year olds. Slight male predominance.

Question 34

Wegener Granulomatosis- Dx

Answer 34

positive cytoplasmic (c-) ANCA test

Question 35

Wegener Granulomatosis- Tx

Answer 35

Corticosteroids

Question 36

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

Answer 36

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

Question 37

What is the most common cause of isolated cutaneous vasculitis?

Answer 37

drugs

Question 38

What are clinical manifestations of isolated cutaneous vasculitis?

Answer 38

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

Question 39

Schonlein-Henoch vasculitis- presentation

Answer 39

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

Question 40

Schonlein-Henoch vasculitis- Dx

Answer 40

IgA deposition in vessel walls and normal complement levels.

Question 41

Type I Cryoglobulins- 3 cancers

Answer 41

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

Question 42

Type I Cryoglobulins- Sx

Answer 42

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

Question 43

Type I Cryoglobulins- Lab findings

Answer 43

increased IgM

Question 44

Type II cryoglobulinemia- 3 associated diseases

Answer 44

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

Question 45

Type II cryoglobulinemia- presentation

Answer 45

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

Question 46

Type II cryoglobulinemia- lab findings

Answer 46

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