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26 Cards in this Set

  • Front
  • Back
Inheritance pattern of:

recurrent epistaxis
skin discolorations
mucosal small vessel AVMs
GI bleeds
Autosomal dominant
(hereditary hemorrhagic telangiectasias = Osler-Weber-Rendu syndrome)
Sequelae of tortuous superficial veins from chronically increased venous pressure
poor wound healing
varicose ulcers
What diseases are associated with arteriolar vasospasm in small vessels of fingers and toes in response to cold temperatures or stress?
Raynaud's disease
SLE
CREST syndrome
hemoptysis, cough, dyspnea
hematuria,
perforation of nasal septum, chronic sinusitis,
otitis media, mastoiditis,
pyoderma gangrenosum
Wegener's granulomatosis:
- c-ANCA
- nodular densities on X-ray
- cyclophosphamide, steroids
- necrotizing granulomas in lung/ upper airway, necrotizing glomerulonephritis
- affects small vessels
Wegener's granulomatosis
- focal necrotizing vasculitis WITHOUT granulomas
- affects small vessels
- p-ANCA
microscopic Polyangiitis
- vasculitis limited to kidney
primary pauci-immune crescentic glomerulonephritis (few antibodies)
Asthma, sinusitis, skin lesions, wrist/foot drop, p-ANCA
Churg-Strauss syndrome
("allergic granulomatosis" or "angiitis")
granulomatous vasculitis with eosinophilia
ANCA-positive
Churg-Strauss syndrome
("allergic granulomatosis" or "angiitis")
seizures, sensory changes, mental retardation, early-onset glaucoma
leptomeningeal angiomatosis
Sturge-Weber syndrome
- small vessels
nevus flammeus
port wine stain (Sturge-Weber)
palpable purpura on buttocks, legs
arthralgias
intestinal hemorrhage
abdominal pain
melena
Henoch-Schonlein purpura
-small vessels: skin, GI, joints
- follows URI
- IgA immune complexes
Palpable purpura/petechiae
inflamed small vessels
after PCN, phenytoin, sulfa, etc
hypersensitivity vasculitis
Smoker with intermittent claudication
superficial nodal phlebitis
cold sensitivity
severe pain in affected part
autoamputation/gangrene
Buerger's disease = thromboangiitis obliterans
- small and medium vessels
- thrombosing vasculitis --> peripheral ischemia
Asian with fever, conjunctivitis, "strawberry tongue," lymphadenitis, desquamative skin rash
Kawasaki disease
- small and medium vessels
- may develop coronary aneurysm
Treatment for Wegener's granulomatosis
cyclophosphamide
corticosteroids
treatment for Kawasaki disease
IV Ig
Aspirin
treatment for Buerger's disease
smoking cessation
acute necrotizing vasculitis in infants/children
Kawasaki disease
Immune-complex mediated transmural vasculitis with fibrinoid necrosis
polyarteritis nodosa
fever, weight loss, malaise, abdominal pain, melena, headache, myalgia, HTN, neurologic dysfunction, cutaneous eruptions, aneurysms and constrictions
polyarteritis nodosa
- small and medium vessels
- Hep B in 30%
- renal and visceral vessels, NOT lungs
- lesions of different ages
Treatment for polyarteritis nodosa
cyclophosphamide
corticosteroids
Young Asian female with fever, arthritis, night sweats, myalgias, skin nodules, eye problems, weak pulses in upper extremities, abdominal pain/diarrhea, increased ESR
Takayasu's arteritis
- medium and large arteries
granulomatous thickening of aortic arch and/or proximal great vessels and/or mesenteric arteries
Takayasu's arteritis
- medium and large arteries
Elderly female with unilateral headache, jaw claudication, impaired vision, increased ESR,
+/- polymyalgia rheumatica
+/- systemic involvement
temporal arteritis (giant cell arteritis)
- medium and large arteries
- can lead to occlusion of ophthalmic artery
treatment of temporal arteritis
high-dose steroids