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Which is the most common form of vasculitis in USA and Europe?
- Giant cell arteritis
- Affects women more than men 2:1
- Greater than 50 y/o.
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Giant Cell Arteritis (GCA) is also known as?
Temporal arteritis.
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GCA is characterized by?
- Granulmatous inflammation of internal elastic lamina
- Usually affects temporal artery.
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What are the presenting symptoms of GCA?
- Headaches with scalp tenderness
- Changes in vision
- Jaw claudication
- Constitutional symptoms.
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Definitive diagnosis of GCA is made by?
- Temporal artery biopsy showing giant cell infiltrates, fragmentation of intima
- Most patients present with ESR >50mm/h.
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What is the treatment for GCA?
- High dose prednisone
- Angioplasty as last resort.
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Which arteries does Polyarteritis Nodosa (PAN) affect?
Small and medium sized arteries of skin, GI, peripheral nerves, joints, kidneys.
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What does PAN look like, histologically?
Transmural necrotizing inflammation, weakening vessels, aneurysm formation, causing ischemic changes.
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What are some clinical features of PAN?
Palpable purpura, livedo reticularis, necrosis of fingertips, orchitis, peripheral neuropathy, constitutional symptoms.
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What infection is PAN associated with?
HepB: Hep B surface antigen found in 10-50% of cases.
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How do you definitively diagnose PAN?
- Biopsy of accessible tissue
- Focal segmental necrotizing vasculitis of medium sized and small arteries.
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Treatment of PAN?
High-dose corticosteroids, cytotoxic medications (cyclophosphamide) in patients with major organ involvement.
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Buerger's disease is also known as?
Thromboangitis obliterans.
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Buerger's disease is strongly associated with?
- Heavy tobacco smoking
- Men 20-45 y/o.
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How does Buerger's present?
Younger patients, distal extremety ischemia, pain at rest, claudication, raynaud's phenomenon, persisten ischemic ulcers.
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Treatment of Buerger's?
Complete discontinuation of smoking.
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Takayasu's arteritis (TA) is also known as?
Pulseless arteritis.
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TA affects, how?
Granulomatous inflammation of large arteries: Aorta and its branches.
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The morbidity and mortality of TA arises from what?
Causes stenosis, thrombosis, or aneurysm in aorta and branching large arteries.
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In whom does TA present, how?
- Young women (teens to thirties), particularly asian
- Fever, malaise, weight loss.
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Diagnosis for TA?
- Clinical: more than 30mmHg difference in BP
- Diagnostic: arteriography, MRA
- No biopsies.
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Treatment for TA?
- High-dose systemic corticosteroids
- Methotrexate and cyclophosphamide in non-responsive patients.
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What is Wegener's Granulomatosis (WG)?
- Systemic vasculitis with granulomatous involvement of small vessels
- Necrosis of upper and lower respiratory tracts
- glomerulonephritis.
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In whom does WG present?
All ages, sexes, and races (somewhat more common in whites).
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Diagnosis of wG?
- c-Anca antibodies
- Anti-proteinase 3 antibodies
- also: biopsy of small vessels show focal necrosis and granulomatous changes.
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What is Kawasaki's disease?
- Febrile vasculitis: Fever, rash, conjunctivitis, strawberry tongue, desquamation of extremities, fissured lips
- Worst symptom: Coronary artery aneurysms.
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Who get's Kawasaki's disease?
- Japanese children less than two (slight male predominance)
- Etiology unknown: infectious and autoimmune.
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Treatment of Kawasaki's?
- High dose aspirin and IV-Ig for inflammation
- Anti-coagulant for coronary symptoms.
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What are the characteristics of Henoch-Shonlein Purpura (HSP)?
- Small vessel vasculitis
- palpable purpura, colicky pain, melena, arthritis, hematuria
- self limited
- median age 4 y/o, 1.5:1 male predomoninance
- Follow URI.
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What are the histologic findings in HSP?
IgA, C3, and immune complex deposition in small vessels (capillaries, arterioles, venules).
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