Pathology: Vascular Diseases W Pics

Front 1

Discuss Arteriovenous Fistulas:
congenital--
acquired--

Back 1

*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.

Front 2

What are the categories of IF staining with ANCA? What is each one useful for?

*c-ANCA
*p-ANCA

Back 2

*c-ANCA – cytoplasmic staining:
1) Wegener granulomatosis.

*p-ANCA – perinuclear staining:
1) microscopic polyangiitis.
2) Churg-Strauss syndrome.

Front 3

Discuss Giant Cell (Temporal) Arteritis in general:

Back 3

*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.

Front 4

Gross and Histologic traits of Giant Cell (Temporal) Arteritis:

Back 4

*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.

Front 5

Back 5

L: Temporal artery. Giant cell pointed to by arrow.
Middle: Elastic stain. IEM is damaged.

*Giant Cell (Temporal) Arteritis.

Front 6

Back 6

*Lumen of artery at left. Arrow points to giant cell.
*Note granuloma and inflammatory cells.
*Giant Cell (Temporal) Arteritis.

Front 7

Discuss Takayasu Arteritis in general:
who does it affect?

Back 7

*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.

Front 8

Back 8

*Takayasu Arteritis
*Arrows point to constrictions.
*Usually multiple constrictions.

Front 9

Gross and Histologic traits of Takayasu Arteritis:

Back 9

*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).

Front 10

Back 10

*Takayasu Arteritis.
*Note swollen appearance of vessel on right.
*Histology is vague; can't distinguish from Giant Cell Arteritis.

Front 11

Back 11

*Takayasu Arteritis.
*Note adventitial inflammation. Intima and media can get kind of wrinkly and funny, but are not as affected as the adventitia.

Front 12

Back 12

*Takayasu Arteritis.
*Note giant cell.

Front 13

Back 13

*Takayasu Arteritis.

Front 14

Discuss Polyarteritis Nodosa (PAN) in general:

Back 14

*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.

Front 15

Back 15

*Skin involvement and wrist drop due to nerve involvement in Polyarteritis Nodosa.

Front 16

Back 16

*Polyarteritis Nodosa: Acute Phase:
-Transmural infiltration by polys, eosinophils, and lymphocytes.
-Fibrinoid necrosis of vessel wall.
-Note sparing of portion of circumference.

Front 17

Back 17

*Polyarteritis nodosa: segmental involvement with portion of vessel wall preserved.

Front 18

Back 18

*Polyarteritis nodosa – kidney (not a glomerulonephritis).
*Arrow denotes artery with huge amount of inflammation around it. Glomeruli are visible and are NOT affected.

Front 19

Back 19

*Polyarteritis Nodosa; Healed Lesion:
-Wall has been replaced by cellular connective tissue.
-Lumen is small and eccentric.

Front 20

Discuss Kawasaki Disease in general:
AKA?
Who does it affect??
What is it associated with?

Back 20

*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.

Front 21

Kawasaki Disease:
Discuss pathological changes--

Back 21

*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!

Front 22

Back 22

*Kawasaki Disease: Cross-section of an aneurysmal coronary artery with occlusive thrombus. 6 month old child with acute, massive MI.

Front 23

Discuss Microscopic Polyangiitis in general:

Back 23

*Small vessel vasculitis.
*Restricted to arterioles, capillaries, and venules.

*Lung involved.

*Kidney involved; necrotizing glomerulonephritis.

Front 24

Histology and ANCA association of Microscopic Polyangiitis:

Back 24

*Histology:
-Wall infiltrated with neutrophils.
-Fibrinoid necrosis.
-No granulomas.

*Strong association with p-ANCA.

Front 25

Back 25

*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.

Front 26

Discuss Churg-Strauss Syndrome in general:

Back 26

*Small vessel vasculitis.

*Patients have peripheral blood eosinophilia.
*Likely to involve respiratory system (asthma).

*Strong association with p-ANCA.

Front 27

Back 27

*Churg-Strauss syndrome.
*Lung tissue. Some alveoli (arrow) have dense accumulations of inflammatory cells. Eosinophils and neutrophils.

Front 28

Back 28

*Churg-Strauss Syndrome.
*Fibrinoid necrosis of artery.
*Bordered by granulomatous inflammation.
*Surrounded by eosinophils.

Front 29

Back 29

*Churg-Strauss Syndrome.
*Bordered by granulomatous inflammation.

Front 30

Discuss Wegener Granulomatosis in general:

Back 30

*Small vessel vasculitis.
*Small to medium-sized arteries and veins.
*Respiratory and renal involvement.

*Strong association with c-ANCA.

Front 31

What distinguishes Wegener Granulomatosis?

Back 31

*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.

*C-ANCA

Front 32

Back 32

*Wegener Granulomatosis.
*Large, nodular, caseous lesions in the lung. Look an awful lot like TB.
*Similar lesions develop in the upper respiratory tract.

Front 33

Back 33

*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).

Front 34

Back 34

*Wegener Granulomatosis.
*Granulomatous inflammation adjacent to small artery vasculitis.
*Giant cells at arrows.

Front 35

Discuss Thromboangiitis Obliterans:

Back 35

*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.

Front 36

Back 36

*Thromboangiitis Obliterans.
*A.K.A. Buerger disease; a disease of young smokers.

Front 37

Back 37

*Thromboangiitis obliterans with microabscess in vessel wall.
*Note elastic lamina.

Front 38

Back 38

*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.

Front 39

Describe Vasculitis in other diseases:
arthritis--
SLE--
others--

Back 39

*Rheumatoid arthritis: small and medium arteries; occasionally aorta.

*Lupus erythematosis or related lupus anticoagulant with antiphospholipid antibody.

*Malignancy.

*Mixed cryoglobulinemia.

*Henoch-Schönlein purpura.

Front 40

Back 40

*Epicardial coronary artery with organized thrombus following vasculitis.
*Young woman with anti-phospholipid antibody (SLE).

Front 41

What's the ∆ b/t true and false aneurysms?

Back 41

*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.

Front 42

Discuss True aneurysms:

Back 42

*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.

Front 43

Back 43

AAA.

Front 44

How do you fix an AAA?
Where are they?

Back 44

*OR you can graft a repair.
Below Renal arteries!

Front 45

Back 45

L: Aneurysm of the brachial artery.
Mid: Multiple thrombi in the lumen.
R: Saphenous vein graft replaces the brachial aneurysm.

Front 46

What are Berry Aneurysms?

Back 46

*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.

Front 47

Discuss Berry Aneurysms:
causes?
pathologic changes?

Back 47

*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.

Front 48

Discuss Syphilitic Aortitis:

Back 48

*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.

Front 49

Back 49

*Syphilitic aortitis - Perivascular Cuffing.

Front 50

Back 50

*Tree Barking in 3˚ syphilis.
*Not diagnostic of lues; can be seen in other vasculitides.
*Lighter part at top is the "tree bark."

Front 51

Discuss Aortic Dissection:

Back 51

*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.

Front 52

Back 52

*Aortic dissection - Intimal tear.
*Aortic valve is visible.

Front 53

Back 53

Front 54

Back 54

Dissection of Aorta to iliac artery.

Front 55

What causes aortic dissection?
How can we catch and prevent it?

Back 55

*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.

Front 56

Back 56

Medial degeneration; precursor of an aortic dissection.

Front 57

Back 57

*Aortic Dissection: Cystic medial degeneration (elastic-trichrome stain).
*Elastic tissue is not orderly.

Front 58

What's the DeBakey classification of aortic dissections?

Back 58

*Deals with which part of aorta is involved (ascending, descending, or both).