Vasculitis

Question 1

Describe Takayasu Arteritis

Answer 1

“aortic arch syndrome”

• affects aorta and main branches (brachiocephalic, left common carotid, left subclavian, carotids)
• granulomatous inflammation of all layers of the blood vessels
• Dx with imaging studies

Question 2

Common syndrome associated with Takauasu Arteritis

Answer 2

Subclavian Steal Syndrome

• stenosis of the left Subclavian vein at its aortic origin “steals” blood from the left vertebral artery to supply the left arm.
• results in syncope or other neuro manifestations

Question 3

What are the main clinical manifestations of Takayasu Arteritis?

Answer 3

Aortic or Subclavian bruits
Asymmetry of blood pressure (usually 10mmHg difference between brachial BPs)
Absence of a pulse in 1 or more extremities

Question 4

Treatment for Takayasu Arteritis

Answer 4

Glucocorticoids (control inflammation)
Angioplasty or bypass (for sclerotic vessels)
Aortic valve surgery (for progressive aortic regurg)
Treat HTN and heart failure

Question 5

Describe Giant Cell Arteritis

Answer 5

Temporal Arteritis

• usually inflammation of branches of the external carotid artery
• patients can experience Amaurosis Fugax (monocular vision loss)
• scalp tenderness
• jaw/tongue claudication

Question 6

Gold Standard to diagnose Giant Cell Arteritis

Answer 6

Temporal Artery biopsy

normally this isn’t available so an Eleveted Sed Rate and/or elevated CRP is used instead

Question 7

Major treatment goal for Giant Cell Arteritis.

Answer 7

Prevent vision loss: immediate glucocorticoids

Question 8

Describe Behcet Disease

Answer 8

Immune mediated vasculitis of all vessel sizes with concurrent oral and genital ulcers, ocular inflammation, cutaneous, neuro, and GI manifestations.
-associated with HLA-B51

Question 9

Treatment for Behcet Disease

Answer 9

Severe Disease: methotrexate, corticosteroids, INF-alpha

Systemic Disease: immunosuppressive therapy

Question 10

Describe Kawasaki Disease

Answer 10

Medium size vasculitis of infants

-possibly viral trigger of the immune attack on vessel walls

Question 11

What is the criteria to diagnose Kawasaki Disease?

Answer 11

Idiopathic Fever for at least 5 days with 4 out of these 5:

1. Bilateral Bulbar Conjunctival Injection
2. Oral Mucous Membrane Changes
3. Peripheral Extremity Changes
4. Polymorphous Rash
5. Cervical Lymphadenopathy

Question 12

Describe the 3 phases of Kawasaki disease

Answer 12

Acute: fever irritability
Subacute: mucocutaneous changes (eyes, tongue, lips)
Convalescent: desquamation or skin peeling phase with edema

Question 13

Biggest complication of Kawasaki Disease

Answer 13

Coronary Artery Aneurysm

Question 14

Treatment for Kawasaki Disease.

Answer 14

Aspirin, prevents the coronary artery aneurysm

-the actual Kawasaki disease will resolve on its own

Question 15

Describe Polyarteritis Nodosa and the major infectious agent associated with it.

Answer 15

Systemic necrotizing vasculitis of medium and small arteries. Associated with Hepatitis B infection
-spares the lungs

Question 16

Treatment for Polyarteritis Nodosa

Answer 16

Glucocorticoids

Question 17

Describe Wegener Granulomatosis

Answer 17

Necrotizing granulomatous vasculitis involving small vessels of the nasopharynx, lungs, and kidneys.
c-ANCA (+) antibody to PR3-ANCA proteins
-causes pleuritis and saddle nose deformity

Question 18

Goal of Wegener Treatment

Answer 18

Induce remission and prevent relapse using immune modulating drugs.

Steroids, cyclophosphamide, rituximab, methotrexate

Question 19

What is Henoch-Schonlein Purpura?

Answer 19

Vasculitis due to IgA immune complex deposition along vessel walls.

• patients present with palpable purpuric raysh on legs and buttocks
• arthritis, abdominal pain, renal disease

Question 20

Treatment for Henoch-Schonlein Purpura.

Answer 20

Supportive

Symptomatic

Question 21

Trigger for Hypersensitivity Vasculitis.

Answer 21

Type III HS reaction to medication, infection leading to formation of palpable purpura, fever, urticarial, edema, lymphadenopathy

Question 22

How is Hypersensitivity Vasculitis diagnosed and treated?

Answer 22

Dx: low serum complement and elevated SED rate, WBCs seen around vessel wall tissue w/ microscopy

Tx: treat the trigger, use antihistamines or corticosteroids

Question 23

What is Churg-Strauss Syndrome?

Answer 23

Necrotizing granulomatosis inflammation with eosinophils involving mainly small vessels of the lungs and skin.

• pANCA (+)
• associated with allergies, asthma

Question 24

Tx for Churg-Strauss Syndrome

Answer 24

Glucocorticoids w/ or w/o cyclophosphamide

Question 25

Infection associated with Mixed Cryoglobulinemia Vasculitis

Answer 25

Hep C infection