Aneurysms
- intrinsic CT defects:
o Marfan syndrome = fibrillin
o Loeys-Dietz = elastin, collagen I & III
o Ehlers-Danlos = collagen III
o Scurvy = collagen cross-linking
- 2 most important disorder predisposing to aortic aneurysms
o atherosclerosis & HTN
- abdominal aneurysm
o males, increased age, smokers
- thoracic aneurysm
o most commonly assoc. w/ HTN
o Marfan, Syphllis
- aortic aneurysm
o syphilitic aneurysms commonly affect the aortic arch
Aortic dissection
- occurs in
o old men with HTN
o younger men with CT disorder (Marfans)
- MC preexisting lesion = cystic medial degeneration
- initiates with intimal tear
- can cause cardiac tamponade or double barreled aorta
- Type A (MC)
o DeBakey I & II
o ascending aorta
- Type B
o DeBakey III
o distal to subclavian
- excruciating pain in anterior chest, radiates to back
Vasculitis
- immune complex deposition
o drug hypersensitivity – serum sickness
o viral infection – polyarteritis nodosa
o pauci-immune
- anti-neutrophil cytoplasmic antibodies (ANCA)
o MPO-ANCA (pANCA): microscopic polyangitis & Churg-strauss
o PR3-ANCA (cANCA): Wegener granulomatosis
- anti-endothelial cell antibodies
o Kawasaki disease
Giant cell (temporal) arteritis
- MC vasculitis in elderly in US & Europe
- granulomatous inflammation of arteries in head
- facial pain
- tender superficial temporal a.
- ocular symptoms
- segmental nodular intimal thickening
- medial granulomatous inflammation
- elastic lamina fragmentation
- multinucleated giant cells
- screening test = ESR
Takayasu arteritis
- pulseless disease
- granulomatous vasculitis of medium/larger arteries
- ocular disturbances
- transmural fibrous thickening of aorta (aortic arch & great vessels)
- severe luminal narrowing of great vessels
- <50 = takayasu
- >50 = giant-cell
- initial symptoms - nonspecific
Polyarteritis nodosa (PAN)
- systemic; small/medium muscular arteries
- renal & visceral but no pulmonary
- 30% have chronic hepatitis B with HBsAg-HbsAb complexes in affected
vessels
- segmental transmural necrotizing inflammation
- all stages of activity (early-late) coexist in diff. vessels or w/in same
vessel (ongoing and recurrent insults)
- malaise, fever, weight loss, HTN, abdominal pain, melena
- diffuse muscular aches/pains, peripheral neuritis
Kawasaki disease
- aka mucocutaneous lymph node syndrome
- leading cause of acquired <3 disease in children
- affect large to medium sized & small vessels
- coronary a. involvement (aneurysm, rupture, acute MI)
- conjunctival & oral erythema and erosion
- edema of hands/feet
- erythema of palms & soles
- desquamated rash
- cervical lymph node enlargement
- Tx: IV Ig therapy + aspirin
Microscopic angiitis
- necrotizing vasculitis affecting capillaries, arterioles, venules
- MPO-ANCA
- aka hypersensitivity or leukocytoclastic vasculitis
- necrotizing GN in 90%
- pulmonary capillaritis common
- hemoptysis, hematuria, proteinuria, bowel pain, muscle pain, palpable
cutaneous purpura
Churg-Strauss syndrome
- aka allergic granulomatosis and angiitis
- small vessel necrotizing vasculitis assoc. w/ asthma, allergic rhinitis,
lung infiltrates, peripheral eosinophilia, extravascular necrotizing
granulomas
- MPO-ANCA
- cutaneous involvement (palpable purpura)
- GI bleeding, renal disease
- heart involvement in 60%
Wegener granulomatosis
- necrotizing vasculitis w/ triad:
o acute necrotizing granuloma of URT
o necrotizing vasculitis of lungs and URT
o renal dz (crescentic GN)
- PR3-ANCA
Raynaud phenomenon
- vasoconstriction of digital arteries and arterioles
- red white blue changes [order matters, was a bonus]
- primary – young women
- secondary – SLE, Buerger, scleroderma
Thrombophlebitis & phlebothrombosis
- deep legs 90%
- prolonged immobilization
- systemic hypercoagulability predisposes
- Trousseau sign – migratory thrombophlebitis in CA
- Homan sign – pain w/ pressure over affected veins , squeezing calf
muscles, or forces dorsiflexion of foot
- PE serious complication
Superior vena cava syndrome
- usually caused by neoplasms that compress or invade the SVC leading to
marked dilation of the veins of the head, neck, arms, and cyanosis
- bronchogenic carcinoma
- mediastinal lymphoma
Inferior vena cava syndrome
- neoplasms that invade or compress or by thrombus leading to marked
LE edema, distension of the superficial collateral veins of the lower
abdomen, massive proteinuria
- HCC
- RCC
Lymphangitis
- acute inflammation elicted when bacterial infection spreads to
lymphatics
- MC = GAS
- red painful streaks, enlargement of lymph nodes
Lymphedema
- primary: congenital or Milroy disease
- secondary: tumors, surgical, irradiation, filiarisis (MC in world), post
radical mastectomy after losing axillary lymph nodes (MC in US)
- Peau d’orange (brawny induration)
- chylous ascities, thorax, pericardium from rupture
Hemangioma
- very common
Buerger disease (thromboangitis obliterans)
- increased amt of normal BVs filled with blood
- segmental, thrombosing, acute & chronic inflammation of medium/small - regress spontaneously
arteries
- angiomatosis – form that involves large portions of body
- affects tibial & radial arteries
- majority are superficial lesions in head & neck
- SMOKING
- 1/3 found in the liver
- superficial nodular phlebitis
- rare malignant transformation
- cold sensitivity of Raynaud
- capillary hemangioma (MC)
- instep claudication
o skin, subQ tissues, mucous membranes, liver, spleen, kidney
- severe pain at rest
o strawberry type (juvenile) is very common in newborns; fades a 1- chronic ulceration of toes, feet, fingers
3 years and complete regression by 7
- cavernous hemangioma
o large dilated channels
o less circumscribed
o can involve deep structures
o assoc. w/ von Hippel-Lindau (involves cerebellum, brain stem,
retina)
o red-blue soft spongy mass 1-2 cm
o large cavernous blood-filled vascular spaces
- pyogenic granuloma
o rapidly growing pedunculated red nodule
o bleeds easily, often ulcerated
o pregnancy tumor (in gingiva)
o assoc. w/ trauma, proliferating capillaries, inflammation,
exuberant granulation-like tissue
Lymphangioma
- simple (capillary)
o head, neck, axillary, subQ tissue
- cavernous (cystic hygromas)
o neck and axilla of children
o Turner syndrome
Glomus tumor (glomangioma)
- benign, very painful, modified smooth m. cells of the glomus body
- distal portion of digits, esp. under the fingernails
Vascular ectasia
- nevus flammeus
o birth mark
o port wine stain
o Sturge-Weber syndrome = port wine stain in trigeminal nerve
distribution; mental retardation
- spider telangiectasia
o hyper-estrogenic states (pregnancy or cirrhosis)
- hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu dz)
o widely distributed throughout body
o rupture can cause serious epistaxis, GI bleed, or hematuria
Bacillary angiomatosis
- vascular proliferation resulting from opportunistic infection
- 1st seen in AIDS
Kaposi sarcoma
- HHV-8; sexually transmitted
- requires cofactor for tumor progression
- 4 forms:
o chronic KS (classic or European)
 old men; Ashkenazi Jew, Mediterranean
 red to purple skin plaques in distal LE
 slowly increase in size and #
 patch  plaque  papule (neoplastic)
o lymphadenopathic KS (African or endemic)
 prevalent in S. African Bantu children
 not assoc. w/ HIV
 skin lesions sparse
 pts present w/ lymphadenopathy
 if tumor involves viscera = extremely aggressive
 MC tumor in central Africa
o transplant associated
 aggressive/fatal
 nodal, mucosal, visceral involvement
o AIDS associated (epidemic)
 most prevalent malignancy in AIDS
Angiosarcoma
- malignant endothelial neoplasms
- older adults more commonly affected; men=women
- usually involve skin, soft tissue, breast, liver
- hepatic angiosarcoma – assoc. w/ exposure to carcinogens (arsenic,
Thorotrast, polyvinyl chloride)
- can arise in setting of lymphedema in UE after radical mastectomy
- can be radiation induced
- all degrees of differentiation can be seen microscopically
- locally invasive; metastasize readily, aggressive
Angioplasty
- abrupt reclosure may occur
- long term can cause proliferative restenosis due to intimal thickening
Vascular replacement
- act like a vascular injury  stenosis … etc.