Wednesday, July 20, 2011

CARDIOVASCULAR PATHOLOGY (vasculitis small-medium-large vessels).

Telangiectasia
Dilated vessels on skin and mucous membrane.
Osler-Weber-Rendu Syndrome
(autosomal dominant inheritance-nosebleeds and skin discoloration)
Affects small vessels
Raynaud’s disease
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.
Small vessels
Wegener's granulomatosis
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.

Small vessels
Microscopic polyangeitis
Like Wegener’s but lacks granulomas
*p-ANCA
Small vessels
1º pauci-immune crescentic glomerulonephritis 
Limited to the kidneys
Small vessels
Churg-Strauss syndrome
Granulomatous vasculitis with eosinophilia.
Involves lungs, heart, skin, kidneys, and nerves.
Often seen in atopic patients.
*p-ANCA


Small vessels
Sturge-Weber disease
Congenital vascular disorder.
Port-wine stain on face and leptomeningeal angiomatosis (intracerebral AVM)
Small vessels



Henoch-Schönlein purpura
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
Small vessels.
Common triad
-skin
-joints
-GI
Buerger’s disease
(thromboangiitis obliterans)
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
Small and medium vessels.
Cause thrombosis/
Infarction of arteries.

Kawasaki disease
Acute, self-limiting disease of infants/kids.
May develop coronary aneurysms.
Fever, congested conjunctiva, strawberry tongue, lymphadenitis.
Necrotizing vasculitis of small and medium vessels.
Polyarteritis nodosa
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.
Necrotizing immune complex inflammation of medium sized muscular arteries. Lesions at different ages.
Takayasu’s arteritis
(pulseless disease)
Asian females <40 years old.
Fever, arthritis, night sweats, myalgia, skin nodules, ocular disturbances, weak pulse in upper extremities.
Granulomatous thickening of aortic arch and
/or proximal great vessels.







Temporal arteritis
(giant cell arteritis)
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.
Medium and large arteries.


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