Creado por Jessica Margaux Mercado
hace alrededor de 9 años
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spherical dilatations; usually seen at bifurcation of the branches of Circle of Willis
direct (abnormal) communication between venous and arterial vessels that bypass the intervening capillary bed
Segments of the vessel wall are focally thickened by a combination of medial and intimal hyperplasia and fibrosis, resulting in luminal stenosis
Neointimal smooth muscle cell growth stimulated by:
Neointimal growth antagonized by:
Basic lesion: fibro-fatty plaque; primarily involves intimal layer of arteries
Atherosclerosis: preferential locations
made up of fat, cellular elements (leukocytes, monocytes, foam cells or macrophages) and fibrous tissues that encloses the central lipid core
Earliest precursor lesion, no clinical significance, may occur early in newborns and children, usually seen from 0-30 y/o;
Flat, yellow spots or streaks; absence of component of smooth muscle cells
Focal intimal plaque with central core of lipid and covering fibrous cap; affects abdominal aorta, coronary, popliteal, descending thoracic aorta, internal carotid, and circle of Willis
A basic lesion becomes altered through:
Calcium deposits in the media (intima if calcified atherosclerosis) of medium-sized muscular arteries (uterine, testicular arteries); >50y/o
Old age, DM; associated with benign hypertension (systolic hypertension)/benign nephrosclerosis; leakage of plasma components across injured endothelium with hyaline deposition by smooth muscle
Homogenous,pink hyaline deposits in media of arterioles; thickening and hyalinization of the walls
Symptoms: renal insufficiency, uremia, moderate reductions in renal plasma flow with normal or slightly reduced GFR, mild proteinuria
Onion-skin arteriole; concentric proliferation of the intimal and smooth muscle cells of an arteriole; occurs in younger pop.;
associated w/ fibrinoid necrosis = necrotizing arteriolitis
Location of Aneurysm: most likely atherosclerotic in nature unless proven otherwise
Location of Aneurysm: syphilitic in nature unless proven otherwise
Location of Aneurysm: developmental and congenital
Location of Aneurysm: Mycotic
Fusiform dilatation associated with severely atherosclerotic aorta; larger aneurysms, >7-8cm in transverse diameter, associated with mural thrombus; majority caused by atherosclerosis; elderly, usually >50y/p
If symptomatic: pulsatile mass, back pain, severe abdominal pain = a sign of imminent rupture; clinically present with a palpable pulsatile mass with bruits and sounds
Complications of AAA
Typically occur in younger patients who often present with back pain and elevated inflammatory markers;
Cause: presumed localized immune response to the abdominal aortic wall;
May be a vascular manifestation of immunoglobulin G4 (IgG4)-related disease
Type of AAA: Lesions that have become infected by the lodging of circulating microorganisms in the wall
complication of tertiary syphilis with obliterative endarteritis of vasa vasorum → tree barking of aorta (intimal destruction)
Complications of Syphilitic Aneurysm
Tree barking of aorta; superimposed atherosclerosis; 2˚ AV regurgitation and dilatation, LV hypertrophy;
obliterative endarteritis of vasa vasorum (due to surrounding inflammation) → diminished blood supply to the blood vessel → progressive dilatation
Occurs when blood separates the laminar planes of the media to form a blood-filled channel within the aortic wall
Sudden, severe unbearable pain either located in the chest (thoracic aneurysm) or in the abdomen or back (abdominal aneurysm)radiating to the back and downwards
Intimal tear → Medial cleavage by dissecting hematoma → “Double-barreled aorta“ → Cystic medial degeneration or necrosis
General term for inflammation of blood vessel wall
Common pathogenic mechanisms of vasculitis
Large Vessel Vasculitis
Medium Vessel Vasculitis
Small Vessel Vasculitis: ANCA Positive
Small Vessel Vasculitis: ANCA Negative
Formation of Ag-Ab complexes and subsequent deposition in blood vessels
group of autoantibodies directed against azurophil or primary granules of neutrophils, lysosomes of monocytes, and endothelial cells
cytoplasmic location of immunofluorescence stain; also known as Anti-proteinase 3 or PR3-ANCA; associated with Wegener granulomatosis
perinuclear location of immunofluorescence stain; also known as Anti-myeloperoxidase or MPO-ANCA; associated with microscopic polyangiitis, PAN, and Churg-Strauss syndrome
Antibodies to endothelial cells, perhaps induced by defects in immune regulation; associated with Kawasaki disease
Most common vasculitis in elderly females; Chronic, granulomatous inflammation that affects small and medium sized arteries in the head and neck especially temporal arteries
T-cell mediated immunologic reaction against the arterial wall leading to a granulomatous reaction;
S/S: Unilateral throbbing headache (affected temporal artery), facial pain, ocular symptoms
Patients may also have systemic manifestations like muscle pains/aches that appears to be rheumatic in nature called polymyalgia rheumatica
Gross: nodular intimal thickening of the involved artery that reduces the luminal diameter;
Histology: medial granulomatous inflammation w/ multinucleated giant cells that leads to internal elastic lamina fragmentation
Granulomatous vasculitis of medium and large arteries (aorta);
Common in women, < 40 yrs. Old
Ocular disturbance (visual defects, retinal detachment, blindness);
Pulseless disease in the upper extremities (if the brachiocephalic artery is involved)
involves aortic arch plus tributaries and branches; Irregularly thickened intima (hyperplasia), fibrous aortic wall, narrowed lumen due to inflammation = weakened peripheral pulse
granuloma with giant cells, similar to temporal arteritis; patchy necrosis of the media of aorta (medial necrosis); may lead to collagenous scarring; aortic involvement may cause aortic valve insufficiency
Transmural (from adventitia to intima) vasculitis of small or medium muscular arteries (not including arterioles, capillaries, or venules); occurs in young, male adults
Acute Phase: transmural neutrophilic inflammation w/ fibrinoid necrosis & thrombotic occlusion;
Healing Phase: fibrous thickening & mononuclear inflammation;
Healed Phase: marked fibrosis w/ minimal inflammation & nodular consistency
Necrotizing vasculitis; affects arterioles, capillaries and venules; all lesions are of the same histologic age; ANCA positive in majority of cases
Associated with necrotizing glomerulonephritis and pulmonary capilliritis;
Associated with drug hypersensitivity (leukocyte cytoplastic vasculitis)
Hemoptysis; hematuria, and proteinuria; bowel pain or bleeding, muscle pain or weakness; and palpable cutaneous purpura
Segmented fibrinoid necrosis of the media with focal transmural necrotizing lesions; Little or no immunoglobulin can be seen in most lesions = Pauci-immune injury
affects large and medium sized arteries with mucocutaneous syndrome; leading cause of acquired heart disease in children; results from delayed hypersensitivity reaction of T- cells
Young children and infants with coronary artery involvement → aneurysms → rupture of thrombus → Acute MI
T-cell and macrophage activation → cytokine production and macrophage activation; polyclonal B cell activation → formation of autoantibodies to endothelial and smooth muscle cells → Acute vasculitis
S/S: fever; mucocutaneous lymph syndrome (conjunctival and oral erythema and erosion, erythema of the palms and soles, edema of the hands and feet, desquamated rash and cervical lymphadenopathy)
Segmented, necrotizing, fibrinoid (less prominent), panarteritis; inflammation affecting entire thickness of vessel wall; proliferation of autoantibodies
Triad:
Acute necrotizing granulomas of URT/LRT,
Focal necrotizing granulomas and vasculitis of small and medium sized arteries,
Proliferative / crescentric glomerulonephritis
Presence of crescents in the Bowman’s capsule of the glomerulus, related to the proliferation of the epithelial cells of the Bowman capsule → results to obliteration
persistent pneumonitis, cavitation, sinusitis, mucosal erosions of RT, renal disease, fever, muscle pains; necrotizing granulomas of nose, palate, pharynx, lungs
Necrotizing, granulomatous vasculitis of small arteries, veins and capillaries;
Renal lesions: Focal necrotizing GN, Crescentic GN
Destruction of mucous membranes in the nose and lips due to vasculitis, Aphthous stomatitis, Saddle nose deformity, Pulmonary nodules
c-ANCA: speckled pattern in cytoplasm
Segmental, acute and chronic inflammation of medium and small arteries, occasionally veins and nerves; accompanied by luminal thrombosis; associated w/ heavy cigarette smoking, usually seen exclusively in middle-aged (and also young) males
intermittent claudication, pain in the lower extremities, ischemic pain, and then suddenly gangrene sets in, in the lower extremities or in the distal digits, sometimes they may present with autoamputation
Abnormal dilatation and tortuosity of the veins
Caused by the venous dilatation of the anal venous plexus
Affects superficial veins of the upper and lower extremities; caused by a prolonged increase intraluminal pressure and loss of vessel support due to the weakening of vein walls
Renders the venous valves incompetent and leads to lower extremity stasis, congestion, edema, pain, and thrombosis
Complications of Varicose Veins
Acute inflammatory process caused by bacterial seeding of the lymphatic vessels; Affected lymphatics are dilated and filled with an exudate of neutrophils and monocytes
Manifested by red, painful subcutaneous streaks and painful enlargement of the draining lymph nodes
Due to the blockade of lymphatics by filarial worms which mainly affects fatty channels;
Associated with fibrosis of the lymph nodes -> blockade -> lymphedema
most common vascular tumor; tumor of pediatric age group; most regress spontaneously
Most common variant; tends to be superficially located: skin, subcutaneous tissue, mucous membranes of oral cavities and lips; spontaneously regress (75%-90% regression through time); luscious red in appearance
Gross: bright red to blue, flat, elevated or pedunculated lesion;
Histology: lobular proliferation of closely packed, thin-walled blood filled capillaries lined by flattened endothelium.+/- thrombosis
Composed of large, dilated vascular channels; larger, more infiltrative; no regression because they are deeply situated
systemic hemangioma in the cerebellum, brain stem, eye, pancreas, and liver
mass is unencapsulated, has infiltrative borders, and is composed of large, cavernous blood-filled vascular spaces separated by connective tissue stroma
pseudotumor; present as rapidly growing red pedunculated lesions on the skin, gingival, or oral mucosa; bleed easily and often ulcerated; 1/3 develop after trauma; associated with acute and chronic inflammation
benign lymphatic counterpart of hemangiomas; proliferation of blood vessels devoid of blood inside; occurs in the subcutaneous tissue, head, axilla, and neck (can be large in the pediatric age)
notable lateral neck cystic mass; nothing more than a lymphangioma
benign, but extremely painful; arise from glomus bodies; common site: sub-ungal
Characterized by localized dilation of preexisting vessels
most common form of ectasia; usual location : head and neck (superficial);
flat, light pink to purple irregular lesion;
majority ultimately fade and regress spontaneously thru time
only dilation of vessels (with normal volume) in the dermis which are non-neoplastic
May be associated with the Sturge-Weber syndrome aka encephalotrigeminal angiomatosis with distribution along trigeminal nerve
Multiple venous angiomatous masses in the leptomeninges and cortex;
Port wine nevi in ipsilateral face;
Mental retardation, seizures;
Neurologic abnormalities (i.e. hemiplegia, and radiopacities in the skull)
Radial, pulsatile network of dilated subcutaneous arteries which blanches on pressure at the center; usual location: face, neck, or upper chest; associated with pregnancy and cirrhosis
Autosomal dominant; genetic malformations associated with dilated cap & veins; widely distributed: skin, mucous membranes of the oral cavity, lips, and respiratory tract, GIT, and urinary tract
Most common AIDS associated cancer;
Scaly very red, elevated with irregular and varying sizes on the skin
Commonly seen (90%) in older men of Eastern European (esp. Ashke-nazi Jews) or Mediterranean descent;
multiple red to purple skin plaques or nodules in the arms or legs
South Africa , young Bantu children; skin and visceral lesions; local or general lymphadenopathy, skin lesions are sparse; aggressive clinical course
Occurs following organ transplant recipient with high doses of immunosuppressive therapy; skin is involved to a lesser extent; aggressive and involves lymph node, mucosa, and visceral organs (primarily involved)
Skin lesions : 3 stages : multiple red (hemorrhagic), purple patch, plaque, and macules in the extremities
malignant vascular tumor;
common site: skin, soft tissue, breast, liver;
etiogenesis: carcinogenic (arsenic, thorotrast, polyvinyl chloride), chronic lymphedema, radiation, foreign body; clinical behavior: local invasion and high risk for distal metastasis, poor outcome, aggressive
Histology: poorly developed vascular channels with red blood cells in the slits, lined by atypical looking (malignant) hyperchromatic spindle cells
Malignant vascular tumor with characteristic thin-walled branching ("staghorn") vascular pattern microscopically
Histogenesis : pericytes;
Anastomosing vascular channels lined by benign looking endothelial cells;
staghorn pattern