Disorder
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Vasculitis
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Epidemiology/Etiology
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Clinical/Laboratory
Findings
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Takayasu's
arteritis ("pulseless disease")
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Granulomatous large
vessel vasculitis involving aortic arch vessels
|
Young Asian women
and children
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Absent upper extremity
pulse
Visual defects, stroke
|
Giant
cell (temporal) arteritis
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Granulomatous large vessel vasculitis
involving superficial temporal and ophthalmic arteries; thrombi contain
microabscesses
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Adults > 50 years of age
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Temporal headache, jaw claudication
(pain when chewing)
Blindness on ipsilateral side
Polymyalgia rheumatica (muscle and joint pain; normal serum creatine kinase)
Increased ESR
|
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Necrotizing
medium-sized vessel vasculitis involving renal, coronary, mesenteric arteries
(spares pulmonary arteries)
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Middle-aged men
Association with HBsAg (30%)
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Vessels at all
stages of acute and chronic inflammation
Focal vasculitis produces aneurysms (detected with angiography)
Organ infarction in kidneys (renal failure), heart (acute MI), bowels (bloody
diarrhea), skin (ischemic ulcer)
|
|
Necrotizing medium-sized vessel
vasculitis involving coronary arteries (e.g., thrombosis, aneurysms)
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Children < 4 years of age
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Desquamating rash, swelling of hands
and feet, cervical adenopathy, oral erythema
Abnormal ECG (e.g., acute MI)
Corticosteroids contraindicated (danger of vessel rupture)
|
Thromboangiitis
obliterans (Buerger's disease)
|
Medium-sized vessel
vasculitis with digital vessel thrombosis
|
Men 25-50 years of
age who smoke cigarettes
|
Foot claudication,
Raynaud's phenomenon, ulceration, gangrene
|
|
Medium-sized vessel vasculitis
involving digital vessels in fingers and toes
|
Young women
Exaggerated vasomotor response to cold or stress
|
Paroxysmal digital color changes
(white-blue-red sequence)
Ulceration and gangrene in chronic cases
|
|
Medium-sized vessel
vasculitis involving digital vessels in fingers and toes
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Adult men and women
Secondary to other diseases (e.g., systemic sclerosis, CREST syndrome)
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Systemic sclerosis
and CREST syndrome: digital vasculitis with vessel fibrosis, dystrophic
calcification, ulceration, gangrene
|
|
Necrotizing medium-sized and small
vessel vasculitis involving upper respiratory tract, lung, renal vessels
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Childhood to middle age
|
Necrotizing vasculitis in upper
respiratory tract (nasopharynx, sinuses, trachea), lower respiratory tract
(pulmonary vessels; infarction, pneumonia), kidneys (crescentic
glomerulonephritis)
|
|
|
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Necrotizing
granulomas in upper respiratory tract (saddle nose deformity), lungs c-ANCA
antibodies (>90% of cases)
Treatment: corticosteroids, cyclophosphamide
|
|
Small vessel vasculitis involving
skin, lung, brain, GI tract, and postcapillary venules and glomerular
capillaries
|
Children and adults
Precipitated by drugs (e.g., penicillin), infections (e.g., streptococci),
immune disorders (e.g., SLE)
|
Vessels at same stage of inflammation
Palpable purpura, glomerulonephritis p-ANCA antibodies (>80% of cases)
|
|
Small vessel
vasculitis involving skin, lung, heart vessels
|
Children and adults
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Allergic rhinitis,
asthma
p-ANCA antibodies (70% of cases), eosinophilia
|
|
Small vessel vasculitis involving
skin, GI tract, renal, joint vessels
|
Children and young adults
Most common vasculitis in children
IgA immunocomplexes
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Palpable purpura of buttocks and
lower extremities
Polyarthritis, glomerulonephritis, GI bleeding
|
|
Small vessel vasculitis
involving skin, GI tract, renal vessels
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Adults
Association with HCV, type I MPGN
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Cryoglobulins:
immunoglobulins that gel at cold temperatures
Palpable purpura, acral cyanosis of nose and ears and Raynaud's phenomenon
(reverses when in warm room)
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|
Small vessel vasculitis involving
immunocomplex deposition in skin vessels
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Children and adults
Complication of treatment of rattlesnake envenomation with horse-or
sheep-based antivenin
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Fever, urticaria with vasculitis,
arthralgia, GI pain with melena
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|
Small vessel
vasculitis involving skin vessels
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Children and adults
Involves all microbial pathogens
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Rocky Mountain
spotted fever: tick transmission of Rickettsia rickettsiae; organisms
invade endothelial cells; petechiae on palms spread to trunk
Disseminated meningococcemia due to Neisseria meningitidis
Capillary thrombosis produces hemorrhage into skin and confluent ecchymoses
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