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70 Cards in this Set
- Front
- Back
Discuss Arteriovenous Fistulas:
congenital-- acquired-- |
*Congenital – especially important in the brain!
-Symptoms will be due to pressure or rupture. *Acquired: -Trauma. -Breakdown of walls: 1) Tumors 2) Infection (abscess). -Iatrogenic: 1) Intentional as in dialysis patients. 2) Unintentional as a postoperative or post catheterization complication. |
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List the types of Vasculitides: 10
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*Giant cell (temporal) arteritis
*Takayasu arteritis *Polyarteritis nodosa *Kawasaki disease *Microscopic polyangiitis/polyarteritis *Churg-Strauss syndrome *Wegener granulomatosis *Thomboangiitis obliterans *In collagen vascular diseases *Infectious |
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Discuss Vasculitis in general:
etiology-- noninfectious vasculitis-- |
*Etiology:
1) Infectious, noninfectious, or unknown. 2) Noninfectious vasculitides: -Referred to as systemic necrotizing vasculitides. -Most are due to immunological mechanisms. -Classified by the size of involved vessels. *Mostly we are talking about arteritis but veins can also be involved. |
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Vasculitis: what's the evidence for an immunological role?
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*Similar lesions seen in experiments involving immune-complex mediation.
*Immune reactants, complement found in serum of symptomatic patients: -DNA-anti-DNA immune complexes in SLE. -IgG, IgM, and complement are elevated in cryoglobulinemic vasculitis. *Hypersensitivity to drugs is implicated in 10% of vasculitic skin lesions: biopsies demonstrate complexes deposited in vessel walls. *Viral infection-associated vasculitis: -HepB surface antigen (HBSAg) and HBsAg-anti-HBsAg immune complexes, with complement, in serum and in vascular lesions of those patients. -Vasculitis will REMIT with immunosuppression, even if the infection persists. *Glomerulonephritis (a vasculitis!) in chronic HCV infection: -HCV/RNA and cryoprecipitates with anti-HCV antibodies in glomeruli. |
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Discuss Antineutrophil Cytoplasmic Antibodies (ANCA):
why are they useful? |
*A heterogeneous group of autoantibodies.
*React to enzymes in azurophilic granules in neutrophils (and related enzymes in other cells). *They are useful diagnostic markers in small vessel vasculitides, via indirect immunofluorescence. |
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What are the categories of IF staining with ANCA? What is each one useful for?
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*c-ANCA – cytoplasmic staining:
1) Wegener granulomatosis. *p-ANCA – perinuclear staining: 1) microscopic polyangiitis. 2) Churg-Strauss syndrome. |
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Describe the process of using ANCA to detect antibodies:
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*Patient’s serum --> incubated with normal neutrophils --> Slides stained using fluorescent-labeled anti-human Ig.
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How are Vasculitides diagnosed?
3 things to consider. |
*Size of vessels involved.
*Histologic findings. *Clinical presentation. |
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List the Classic Vasculitides:
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*LARGE:
-Giant cell (temporal) arteritis. -Takayasu arteritis. *Medium: -Polyarteritis nodosa. -Kawasaki disease. *small: -Microscopic polyangiitis/polyarteritis. -Churg-Strauss syndrome. -Wegener granulomatosis. |
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Discuss Giant Cell (Temporal) Arteritis in general:
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*Most common vasculitis.
*Classified as a large vessel vasculitis: -Can affect aorta. -Predominantly affects medium-sized and small arteries, especially the cranial arteries. *Disease of older people (>50). *Temporal, opthalmic, facial arteries most commonly affected. |
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Gross and Histologic traits of Giant Cell (Temporal) Arteritis:
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*Gross – nodular swellings (beading), marked narrowing of lumen, thrombi can develop.
*Histology - 2 patterns: 1) granulomatous inflammation in media near internal elastic membrane (IEM), with giant cells and lymphocytes. 2) nonspecific arteritis with no giant cells. |
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L: Temporal artery. Giant cell pointed to by arrow.
Middle: Elastic stain. IEM is damaged. *Giant Cell (Temporal) Arteritis. |
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*Lumen of artery at left. Arrow points to giant cell.
*Note granuloma and inflammatory cells. *Giant Cell (Temporal) Arteritis. |
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Discuss Takayasu Arteritis in general:
who does it affect? |
*Classified as a large vessel vasculitis:
-Affects the aorta. -Also affects the medium-sized branches of the aortic arch. -Occasionally, the pulmonary artery involved. *“Pulseless disease” – Affects the upper extremities! *Mostly affects young women (<40). *In some cases, may result in aneurysmal dilation of ascending aorta, aortic valve regurgitation, dissection. |
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*Takayasu Arteritis
*Arrows point to constrictions. *Usually multiple constrictions. |
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Gross and Histologic traits of Takayasu Arteritis:
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*Adventitial and medial mononuclear infiltrate.
*Perivascular cuffing of vasa vasorum. *Giant cells and foci of medial necrosis. *Histologically, hard to distinguish from temporal (giant cell) arteritis and lues (syphilitic lesions). |
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*Takayasu Arteritis.
*Note swollen appearance of vessel on right. *Histology is vague; can't distinguish from Giant Cell Arteritis. |
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*Takayasu Arteritis.
*Note adventitial inflammation. Intima and media can get kind of wrinkly and funny, but are not as affected as the adventitia. |
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*Takayasu Arteritis.
*Note giant cell. |
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*Takayasu Arteritis.
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Discuss Polyarteritis Nodosa (PAN) in general:
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*Medium vessel vasculitis (medium to small arteries).
*Segmental, not circumferential involvement of arteries. *“Nodules” develop (aneurysmal dilatations). *NO involvement of arterioles (NO glomerulonephritis). *Not usually ANCA positive. *ABSENT or MINIMAL immune complex deposition. *Multiorgan involvement: -KIDNEYS in 85%, HEART in 75% (plus liver in 60%, GI tract in 50%, muscle in 40%, pancreas in 35%, also testes, peripheral nerves , CNS, and skin). *Lungs NOT involved. |
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*Skin involvement and wrist drop due to nerve involvement in Polyarteritis Nodosa.
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*Polyarteritis Nodosa: Acute Phase:
-Transmural infiltration by polys, eosinophils, and lymphocytes. -Fibrinoid necrosis of vessel wall. -Note sparing of portion of circumference. |
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*Polyarteritis nodosa: segmental involvement with portion of vessel wall preserved.
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*Polyarteritis nodosa – kidney (not a glomerulonephritis).
*Arrow denotes artery with huge amount of inflammation around it. Glomeruli are visible and are NOT affected. |
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*Polyarteritis Nodosa; Healed Lesion:
-Wall has been replaced by cellular connective tissue. -Lumen is small and eccentric. |
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Discuss Kawasaki Disease in general:
AKA? Who does it affect?? What is it associated with? |
*Medium vessel vasculitis.
*A.K.A. mucocutaneous lymph node syndrome. *Affects children. *Appears in clusters, may be autoimmune. *Usually associated with fever, oral and conjunctival erythema/erosion, erythema of palms and soles, rash, enlarged cervical lymph nodes. |
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Kawasaki Disease:
Discuss pathological changes-- |
*An acute necrotizing vasculitis of entire wall.
*May involve coronaries, with later development of aneurysms! *Tendency to form occlusive thrombi. *Can result in MI or sudden death. *Due to anti-endothelial cell antibodies! |
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*Kawasaki Disease: Cross-section of an aneurysmal coronary artery with occlusive thrombus. 6 month old child with acute, massive MI.
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Discuss Microscopic Polyangiitis in general:
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*Small vessel vasculitis.
*Restricted to arterioles, capillaries, and venules. *Lung involved. *Kidney involved; necrotizing glomerulonephritis. |
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Histology and ANCA association of Microscopic Polyangiitis:
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*Histology:
-Wall infiltrated with neutrophils. -Fibrinoid necrosis. -No granulomas. *Strong association with p-ANCA. |
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*Cutaneous leukocytoclastic vasculitis: clinically shows as purpura. Polys infiltrate vascular wall and surrounding tissue, with fragmentation of nuclei--> “nuclear dust.”
*Fibrin and immunoglobulin deposition imparts a smudgy appearance. *Small, round, purple dots are fragments of polys. |
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Discuss Churg-Strauss Syndrome in general:
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*Small vessel vasculitis.
*Patients have peripheral blood eosinophilia. *Likely to involve respiratory system (asthma). *Strong association with p-ANCA. |
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Histology in Churg-Strauss Syndrome:
What distinguishes it from polyarteritis nodosa and microscopic polyangiitis? |
*In some ways is similar to polyarteritis nodosa and microscopic polyangiitis:
-Wall infiltration by neutrophils. -Fibrinoid necrosis in vessel wall. *But also has: -Granulomas. -Eosinophils in walls and surrounding tissue. |
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*Churg-Strauss syndrome.
*Lung tissue. Some alveoli (arrow) have dense accumulations of inflammatory cells. Eosinophils and neutrophils. |
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*Churg-Strauss Syndrome.
*Fibrinoid necrosis of artery. *Bordered by granulomatous inflammation. *Surrounded by eosinophils. |
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*Churg-Strauss Syndrome.
*Bordered by granulomatous inflammation. |
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Discuss Wegener Granulomatosis in general:
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*Small vessel vasculitis.
*Small to medium-sized arteries and veins. *Respiratory and renal involvement. *Strong association with c-ANCA. |
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What distinguishes Wegener Granulomatosis?
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*Necrotizing vasculitis.
*Characterized by a triad: -Acute necrotizing granulomas (respiratory tract – upper, lower, or both). -Focal necrotizing, granulomatous vasculitis (most prominent in respiratory tract). -Focal or crescentic glomerulonephritis. *Granulomas are vascular and extravascular. *Must rule out TB and fungal infection. |
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*Wegener Granulomatosis.
*Large, nodular, caseous lesions in the lung. Look an awful lot like TB. *Similar lesions develop in the upper respiratory tract. |
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*Left: Granulomatous inflammation surrounding a blood vessel and a bronchus.
*Mid: Perivascular granuloma (top left) with central necrosis. *Right: A granuloma with central necrosis and giant cell (arrow). |
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*Wegener Granulomatosis.
*Granulomatous inflammation adjacent to small artery vasculitis. *Giant cells at arrows. |
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Discuss Thromboangiitis Obliterans:
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*A.K.A. Buerger disease; a disease of young smokers.
*Segmental acute/chronic inflammation, occasionally with giant cells. *Thromboses in medium and small arteries, especially the radial and tibial arteries. *Inflammation may extend to veins and nerves. |
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*Thromboangiitis Obliterans.
*A.K.A. Buerger disease; a disease of young smokers. |
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*Thromboangiitis obliterans with microabscess in vessel wall.
*Note elastic lamina. |
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*Top: Acute lesion of Buerger’s disease in a vein with intense thromboangiitis, showing a microabscess in the thrombus with two multi-nucleated giant cells (arrows).
*Bottom: Vessel thromboses, neutrophils in wall of artery and adjacent veins. With time, there will be recanalization. |
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Describe Vasculitis in other diseases:
arthritis-- SLE-- others-- |
*Rheumatoid arthritis: small and medium arteries; occasionally aorta.
*Lupus erythematosis or related lupus anticoagulant with antiphospholipid antibody. *Malignancy. *Mixed cryoglobulinemia. *Henoch-Schönlein purpura. |
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*Epicardial coronary artery with organized thrombus following vasculitis.
*Young woman with anti-phospholipid antibody (SLE). |
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Discuss Infectious Arteritis:
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*Vessel walls can be involved in bacterial pneumonia, tuberculous lesions, abscesses, meningitis.
*Focal infectious arteritis with septic emboli or septicemia. *If the wall expands – mycotic aneurysm. *Fungi may also cause infectious arteritis, especially Aspergillus and the organisms of mucormycosis. |
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What's the ∆ b/t true and false aneurysms?
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*True aneurysm: Localized dilation in which all 3 layers of vessel wall or heart are involved.
*False aneurysm: Extravascular hematoma ommunicating with lumen or chamber, due to rupture of wall of vessel or heart chamber. |
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Discuss True aneurysms:
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*Weakened vessel wall is due to genetic or acquired disease.
*Most common cause = atherosclerosis. -Atherosclerosis thins the media. *In aorta, the abdominal portion is most affected: -Is most severely involved by atherosclerosis. -Is the most common site of aneurysm formation. -Abdominal aortic aneurysm - AAA. *Fortunately usually infrarenal. Kidneys get enough blood flow. |
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Shapes of true aneurysms?
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*Saccular –
involve a portion of circumference and are spherical; often filled with thrombus. *Fusiform – shaped like a spindle, tapered at both ends - involve entire circumference. |
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AAA.
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How do you fix an AAA?
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*OR you can graft a repair.
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L: Aneurysm of the brachial artery.
Mid: Multiple thrombi in the lumen. R: Saphenous vein graft replaces the brachial aneurysm. |
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What are Berry Aneurysms?
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*A special group of saccular aneurysms.
*Thin-walled outpouchings at branch points in Circle of Willis; 1-3 mm. *Wide or narrow neck. *Rupture occurs at apex of sac--> can lead to SAH. |
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Discuss Berry Aneurysms:
causes? pathologic changes? |
*Frequent cause of subarachnoid hemorrhage; may involve brain tissue.
*Most cases sporadic. *However, can be associated with: 1) Autosomal dominant polycystic disease (ADPKD). 2) Neuro-fibromatosis (NF) type 2. 3) Ehlers-Danlos syndrome. 4) Marfan syndrome. *Wall of sac shows thick intima with absent or thin fragmented media. |
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Discuss Syphilitic Aortitis:
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*Now rare, it is a prominent feature of tertiary syphilis (lues).
*Begins in adventitia: -obliterative endarteritis of vasa vasorum. -perivascular cuffing - lymphocytes, plasmas. *Medial necrosis with loss of elastic fibers and smooth muscle. *Dissection unlikely; medial fibrosis develops. |
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*Syphilitic aortitis - Perivascular Cuffing.
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*Tree Barking in 3˚ syphilis.
*Not diagnostic of lues; can be seen in other vasculitides. *Lighter part at top is the "tree bark." |
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Discuss Aortic Dissection:
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*Blood enters a defective media through a tear in the intima.
*The media splits into inner and outer layers, for variable distances. *Dissection can continue into the media of branching vessels (i.e. renal arteries from Abd Aorta). *Dissecting blood can re-enter lumen or rupture into surrounding tissue or body cavity; e.g. pleural or pericardial. |
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*Aortic dissection - Intimal tear.
*Aortic valve is visible. |
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Dissection of Aorta to iliac artery.
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What causes aortic dissection?
How can we catch and prevent it? |
*Hypertension is the leading cause.
*In younger patients, due to rare diseases: -genetically determined defects of collagen, fibrillin or elastin as in Marfan, Turner, or Ehlers-Danlos syndromes. *“Cystic medial degeneration” may be preexisting lesion seen on histology: -Misnomer: elastic tissue fragmentation with replacement by extracellular matrix material. Not truly cystic. |
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Medial degeneration; precursor of an aortic dissection.
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*Aortic Dissection: Cystic medial degeneration (elastic-trichrome stain).
*Elastic tissue is not orderly. |
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What's the DeBakey classification of aortic dissections?
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*Deals with which part of aorta is involved (ascending, descending, or both).
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Discuss complications of Aortic Dissection:
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*Dissection to aortic root distorts aortic valve and causes AV regurgitation.
*Dissection to aortic root with rupture into pericardial sac causes cardiac tamponade. *Dissections can occur de novo in other arteries such as coronary, hepatic, splenic, renal, etc. |
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One more time: 3 key things to bear in mind when diagnosing the Vasculitides:
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-Size of vessels involved.
-Histologic findings. -Clinical presentation. |