Describe Takayasu Arteritis
“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
Common syndrome associated with Takauasu Arteritis
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
What are the main clinical manifestations of Takayasu Arteritis?
Aortic or Subclavian bruits
Asymmetry of blood pressure (usually 10mmHg difference between brachial BPs)
Absence of a pulse in 1 or more extremities
Treatment for Takayasu Arteritis
Glucocorticoids (control inflammation)
Angioplasty or bypass (for sclerotic vessels)
Aortic valve surgery (for progressive aortic regurg)
Treat HTN and heart failure
Describe Giant Cell Arteritis
Temporal Arteritis
- usually inflammation of branches of the external carotid artery
- patients can experience Amaurosis Fugax (monocular vision loss)
- scalp tenderness
- jaw/tongue claudication
Gold Standard to diagnose Giant Cell Arteritis
Temporal Artery biopsy
normally this isn’t available so an Eleveted Sed Rate and/or elevated CRP is used instead
Major treatment goal for Giant Cell Arteritis.
Prevent vision loss: immediate glucocorticoids
Describe Behcet Disease
Immune mediated vasculitis of all vessel sizes with concurrent oral and genital ulcers, ocular inflammation, cutaneous, neuro, and GI manifestations.
-associated with HLA-B51
Treatment for Behcet Disease
Severe Disease: methotrexate, corticosteroids, INF-alpha
Systemic Disease: immunosuppressive therapy
Describe Kawasaki Disease
Medium size vasculitis of infants
-possibly viral trigger of the immune attack on vessel walls
What is the criteria to diagnose Kawasaki Disease?
Idiopathic Fever for at least 5 days with 4 out of these 5:
- Bilateral Bulbar Conjunctival Injection
- Oral Mucous Membrane Changes
- Peripheral Extremity Changes
- Polymorphous Rash
- Cervical Lymphadenopathy
Describe the 3 phases of Kawasaki disease
Acute: fever irritability
Subacute: mucocutaneous changes (eyes, tongue, lips)
Convalescent: desquamation or skin peeling phase with edema
Biggest complication of Kawasaki Disease
Coronary Artery Aneurysm
Treatment for Kawasaki Disease.
Aspirin, prevents the coronary artery aneurysm
-the actual Kawasaki disease will resolve on its own
Describe Polyarteritis Nodosa and the major infectious agent associated with it.
Systemic necrotizing vasculitis of medium and small arteries. Associated with Hepatitis B infection
-spares the lungs
Treatment for Polyarteritis Nodosa
Glucocorticoids
Describe Wegener Granulomatosis
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
Goal of Wegener Treatment
Induce remission and prevent relapse using immune modulating drugs.
Steroids, cyclophosphamide, rituximab, methotrexate
What is Henoch-Schonlein Purpura?
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
Treatment for Henoch-Schonlein Purpura.
Supportive
Symptomatic
Trigger for Hypersensitivity Vasculitis.
Type III HS reaction to medication, infection leading to formation of palpable purpura, fever, urticarial, edema, lymphadenopathy
How is Hypersensitivity Vasculitis diagnosed and treated?
Dx: low serum complement and elevated SED rate, WBCs seen around vessel wall tissue w/ microscopy
Tx: treat the trigger, use antihistamines or corticosteroids
What is Churg-Strauss Syndrome?
Necrotizing granulomatosis inflammation with eosinophils involving mainly small vessels of the lungs and skin.
- pANCA (+)
- associated with allergies, asthma
Tx for Churg-Strauss Syndrome
Glucocorticoids w/ or w/o cyclophosphamide
