Vascular Pathology

Question 1

Arterial wall layers

Answer 1

endothelial intima, smooth muscle media, connective tissue adventitia

Question 2

Vasculitis

Answer 2

Etiology usu unknown;
Clinical features include:
1) nonspecific inflammation (fever, fatigue, weight loss, myalgias)
2) symptoms of organ ischemia - due to luminal narrowing or thrombosis of inflamed vessels
Divided into large (aorta and major branches), medium (muscular arteries that supply organs), small-vessels (capillaries and venules)

Question 3

Temporal Arteritis

Answer 3

Granulomatous vasculitis classically involving carotids
Affects F >50yo
Presents as headache, visual disturbances (opthalmic artery) and jaw claudication; Flu-like symptoms with joint and muscle pain (polymyalgia rheumatica) are often present
Elevated ESR
Biopsy reveals inflamed vessel walls with giant cells and intimal fibrosis; Lesions are segmental and diagnosis requires biopsy of a long segment of vessel
Tx. is corticosteroids

Question 4

Takayasu Arteritis

Answer 4

Granulomatous vasculitis that classically involves the aortic arch at branch points;
Presents in adults <50 (young Asian females)
Visual and neurologic symptoms with weak or absent pulse in upper extremity (pulseless disease)
ESR elevated
Tx. is corticosteroids

Question 5

Polyarteritis Nodosa

Answer 5

Medium vessel vasculitis
Necrotizing vasculitis involving multiple organs except lungs
Classically presents with young adults as hypertension (renal artery involvement), abdominal pain with melena (mesenteric artery involvement), neurologic disturbances and skin lesions
Associated with HBsAg
Early lesions consist of transmural inflammation with fibrinoid necrosis –> fibrosis producing a “string of pearls” appearance
Tx. corticosteroids and cyclophosphamide

Question 6

Kawasaki Disease

Answer 6

Asian children <4 years
Presents with non-specific signs including fever, conjunctivitis, erythematous rash of palms and soles, enlarged cervical lymph nodes
Coronary artery involvement is common and leads to risk for thrombosis with MI and aneurysm with rupture
Tx. is asprin and IVIG

Question 7

Buerger Disease

Answer 7

Necrotizing vasculitis involving the digits
Presents with ulceration, gangrene and autoamuptation of the fingers and toes; Raynaud phenomenon is often present
Highly associated with heavy smoking

Question 8

Wegner Granulomatosis/Granulomatosis with Polyangiitis (GPA)

Answer 8

Small vessel vasculitis
Necrotizing granulomatous vasculitis involving nasopharynx, lungs and kidneys
Classic presentation is middle aged male with sinusitis or nasopharyngeal ulceration, hempotysis and bilateral nodular lung infiltrates and hematuria due to RPGN
Serum c-ANCA (anti proteinase 3) levels correlate with disease activity
Biopsy revels large necrotizing granulomas with adjacent necrotizing vasculitis
Tx. cyclophosphamide and steroids

Question 9

Microscopic Polyangiitis

Answer 9

Necrotizing vasculitis involving multiple organs esp. lungs and kidneys
Presentation is similar to Wegners but nasopharyngeal involvement and granulomas are absent
Serum p-ANCA (anti myeloperoxidase) levels correlate with disease activity
Tx. is corticosteroids and cyclophosphamide

Question 10

Churg-Strauss Syndrome/Eosinophilic Granulomatosis with Polyangiitis

Answer 10

Also called allergic granulomatosis
Necrotizing granulomatous inflammation with eosinophils involving multiple organs esp. lung and heart
Asthma and peripheral eosinophilia often present
Serum p-ANCA levels correlate with disease activity

Question 11

c-ANCA

Answer 11

Wegners/GPA

Anti-proteinase 3 antibodies

Question 12

p-ANCA

Answer 12

Microscopic Polyangitis and Allergic granulomatosis

Anti-myeloperoxidase antibodies

Question 13

Henoch-Schonlein Purpura

Answer 13

Vasculitis due to IgA immune complex deposition
Most common in children
Presents with palpable purpura on buttocks and legs, GI pain and bleeding and hematuria (IgA nephropathy)
Usu occurs following a URTI
Disease is self limited but can recur

Question 14

Secondary HTN

Answer 14

Renal artery stenosis is a common cause
Stenosis decreased blood flow to glomerulus –> JG responds by secreting renin –> ATI –> ATII –> increased blood pressure by contracting arteriolar smooth muscles increasing TPR and promoting adrenal release of Aldo –> increased reabsorption of sodium in DCT
HTN with increased renin and unilateral atrophy (due to low blood flow) of the affected kidney
Stenosis could be due to atherosclerosis or fibromuscular dysplasia (young females)

Question 15

Fibromuscular Dysplasia

Answer 15

Developmental defect of blood vessel wall resulting in irregular thickening of large and medium size vessels; esp. renal; occurs in young females and causes secondary HTN

Question 16

Aortic Dissection

Answer 16

Presents as sharp, tearing chest pain that radiates to back; Occurs in proximal 10 cm of the aorta
Associated with HTN –> hyaline arteriosclerosis of vasa vasorum –> decreased flow that causes atrophy of media
Can also be due to Marfan syndrome or Ehlers-Danlos where there is a weakness of the CT in the media
Complications: pericardial tamponade, rupture with fatal hemorrhage, obstruction of branching arteries with end organ ischemia

Question 17

Thoracic Aneurysm

Answer 17

Ballon like dilation due to weakness in aortic wall
Classically seen in tertiary syphilis; Endarteritis of the vasa vasorum results in luminal narrowing, decreased flow and atrophy of vessel wall –> ‘tree bark’ appearance
Complication is dilation of aortic valve root resulting in insufficiency

Question 18

Abdominal Aortic Aneurysm

Answer 18

Usually arises below the renal arteries but above the bifurcation
Classically seen in M smokers >60yo
Primarily due to atherosclerosis
Presents as pulsatile abdominal mass that grows with time and causes minor abdominal pain
Major complication is rupture when >5cm in diameter - presents with triad of hypotension, pulsatile abdominal mass and flank pain

Question 19

Hemangioma

Answer 19

Benign tumor of blood vessels

Question 20

Angiosarcoma

Answer 20

Malignant proliferation of endothelial cells (CD 31+ - PECAM1 platelet endothelial cell adhesion molecule; expressed on surface of endothelial cells)
Common sites include skin, breast and liver
Liver is associated with exposure to polyvinyl chloride, arsenic and Thorotrast