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