CARDIOVASCULAR PATHOLOGY (vasculitis small-medium-large vessels).
Telangiectasia
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Dilated vessels on skin and mucous membrane.
Osler-Weber-Rendu Syndrome
(autosomal dominant inheritance-nosebleeds and skin discoloration)
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Affects small vessels
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Raynaud’s disease
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Decreased blood flow to the skin due to arteriolar vasospasm in response to cold temperature or emotional stress.
Most often in the fingers and toes.
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Small vessels
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Wegener's granulomatosis
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Necrotizing vasculitis.
Necrotizing granulomas in the lungs and upper airway.
Necrotizing glomerulonephritis.
(Perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis, cough, dysnea, hemoptysis, hematuria).
*c-ANCA
Treatment: Cyclophosphamide and corticosteroids.
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Small vessels
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Microscopic polyangeitis
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Like Wegener’s but lacks granulomas
*p-ANCA
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Small vessels
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1º pauci-immune crescentic glomerulonephritis
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Limited to the kidneys
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Small vessels
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Churg-Strauss syndrome
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Granulomatous vasculitis with eosinophilia.
Involves lungs, heart, skin, kidneys, and nerves.
Often seen in atopic patients.
*p-ANCA
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Small vessels
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Sturge-Weber disease
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Congenital vascular disorder.
Port-wine stain on face and leptomeningeal angiomatosis (intracerebral AVM)
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Small vessels
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Henoch-Schönlein purpura
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Most common form on childhood.
Skin rash on buttocks and legs, arthralgia, intestinal hemorrhage, abdominal pain, and melena.
Follows with URIs.
Associates with IgA nephropathy.
Lesions at the same age
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Small vessels.
Common triad
-skin
-joints
-GI
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Buerger’s disease
(thromboangiitis obliterans)
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Idiopathic, segmental, thrombosing vasculitis.
Seen in heavy smokers.
Intermittent claudication, superficial nodular phlebitis, cold sensitivity (Raynaud’s phenomenon), severs pain in affected part. May lead to gangrene and autoamputation digits.
Treatment: smoking cessation
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Small and medium vessels.
Cause thrombosis/
Infarction of arteries.
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Acute, self-limiting disease of infants/kids.
May develop coronary aneurysms.
Fever, congested conjunctiva, strawberry tongue, lymphadenitis.
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Necrotizing vasculitis of small and medium vessels.
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Polyarteritis nodosa
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Fever, weight loss, malaise, abdominal pain, melena, headache, myalgia, hypertension, neurologic dysfunction, cutaneous eruption.
Hepatitis B + in 30 %
Aneurysms and constrictions on arteriogram.
Treatment: corticosteroids, Cyclophosphamide.
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Necrotizing immune complex inflammation of medium sized muscular arteries. Lesions at different ages.
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Takayasu’s arteritis
(pulseless disease)
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Asian females <40 years old.
Fever, arthritis, night sweats, myalgia, skin nodules, ocular disturbances, weak pulse in upper extremities.
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Granulomatous thickening of aortic arch and
/or proximal great vessels.
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Temporal arteritis
(giant cell arteritis)
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Most common vasculitis that affects medium and large arteries, usually branches of carotid artery.
Focal, Granulomatous inflammation. Affects elderly females.
Unilateral headache, jaw claudication, impaired vision.
↑ ESR, systemic involvement and polymyalgia rheumatic.
Treatment: high doses of steroids.
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Medium and large arteries.
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