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Uncommon Arteriopathies. Vasculitis Temporal arteritis Periarteritis Nodosa SLE Behcet’s disease Kawasaki’s disease Rheumatoid disease with vasculitis Relapsing polychondritis Congenital diseases with arterial involvement Marfan syndrome Ehlers-Danlos syndrome Pseudoxanthoma elasticum

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uncommon arteriopathies
Uncommon Arteriopathies
  • Vasculitis
    • Temporal arteritis
    • Periarteritis Nodosa
    • SLE
    • Behcet’s disease
    • Kawasaki’s disease
    • Rheumatoid disease with vasculitis
    • Relapsing polychondritis
  • Congenital diseases with arterial involvement
    • Marfan syndrome
    • Ehlers-Danlos syndrome
    • Pseudoxanthoma elasticum
    • Homocystinurea
    • Neurofibromatosis
    • Tuberous sclerosis
    • Coarctation of the abdominal aorta
    • Persistent sciatic artery
  • Uncommon acquired arteriopathies
    • Tumor
    • Radiation
    • Focal calcific aortic obstruction
    • Iliac syndrome in cyclists
vasculitis
Vasculitis
  • Acute or chronic inflammatory changes of small, medium, and large arteries or veins
  • Often accompanied by systemic signs of fever, malaise, and weight loss
    • Rheumatologic and cutaneous lesions common
  • Immune mechanisms implicated in almost all entities
    • Triggers of reaction
      • Drugs
      • Infections
      • Inflammation
      • Neoplasia
    • In most cases, however, inciting factor not identified
  • Usually treated with steroids and cytotoxic agents
  • Role of surgery
    • Biopsy
    • removal of necrotic tissue
temporal arteritis
Temporal Arteritis
  • Epidemiology
    • Average annual incidence 17.4 cases per 100,000 patients >50yo
    • Increases with age
      • 1.4x 50-59 yo
      • 10.7x 60-69 yo
      • 29.6x 70-79 yo
      • 28.9x >80 yo
temporal arteritis5
Temporal Arteritis
  • Arterial lesions
    • Characteristic bilateral, symmetric stenosis or occlusion with steady onset over 1-3 months
      • Produces claudication or absence of distal pulses
    • Most common site subclavian-axillary-brachial system
temporal arteritis6
Temporal Arteritis
  • Second most common pattern is bilateral involvement of profunda and SFA
  • Angiography
    • Multiple stenotic areas
    • Post-stenotic dilatation
    • Generous collaterals develop
temporal arteritis7
Temporal Arteritis
  • Clinical picture
    • Typically begins with flu-like illness
      • Malaise
      • Fever
      • Weight loss
      • Scalp tenderness or headache
    • Symptoms intensify over 1-3 weeks
      • Tender, red, or elevated temporal/occipital arteries occurs in 45-60% of patients
      • Jaw claudication 2/3 of patients
    • Eye symptoms occur at about 3 months
      • Blindness, amarosis, extraocular muscle dysfunction
        • Opthalmic, posterior ciliary, retinal vessels
      • “prime medical emergency in opthamology” (Niederkohr et al, Opthamology 112:744, 2005)
    • Large arteries involved 9-14%
      • Death caused by dissection, aneurysm rupture, MI, or stroke
temporal arteritis8
Temporal Arteritis
  • Laboratory Findings
    • Elevated ESR (40-140) in active disease
    • Mild normocytic anemia
    • Mild leukocytosis and thrombocytosis
  • Temporal Artery Biopsy
    • Outpatient procedure under local anesthesia
      • 2cm of vessel
      • <0.5% complication rate
    • 90-99% sensitivity
      • Ultrasound less reliable (Karassa et al, Ann Int Med 142:359, 2005)
        • 60-70% sensitivity
        • Dark halo around temporal artery lumen
  • Therapy
    • Should not be delayed while awaiting biopsy
      • If biopsy negative then withdraw steroids
      • 60 mg/day prednisone, then tapered over subsequent weeks
periarteritis nodosa
Periarteritis Nodosa
  • Most common from 30-50s M>F
  • Focal skip lesions affecting small and medium-sized arteries of all organs
  • Sequence of damage
    • Acute inflammation
    • Necrosis
    • Secondary thrombosis
    • Late fibrosis
  • Major surgical issues involve stenoses of GI/renal beds
    • Lead to cholecystitis, appendictis, GI bleed, ischemic bowel
    • 20 % of patients experience aneurysmal disease of visceral, renal, distal limb vessels
      • Often regress with steroid treatment
  • Steroid therapy increases 5-year survival from 15% in untreated patients to 50%
    • Primary cause of mortality acutely is from renal or GI tract involvement
    • CV and cerebral events in long-term disease
behcet s diseae
Behcet’s Diseae
  • Recurrent aphthous ulcers of mouth/genital tract, uveitis, skin lesions
  • Seen in Asian/mediterranean countries
  • Immune complexes found in vessel walls
    • Treated with cyclosporine, azathiioprine
  • Major arterial/venous lesions occur in 6-25% of cases
    • Leading cause of death
    • Thrombosis and aneurysmal disease (pulmonary)
    • Repair can be done by sewing to healthy vessel tissues
marfan syndrome
Marfan Syndrome
  • Autosomal dominant defect of cross-linking of collagen
    • Mutations of type I procollagen or processing enzymes
  • Tissue changes at autopsy confined to ascending aorta
    • Thickened but weakened
    • Irregular muscle bundle patterns
    • Increased collagen
    • Decreased elastin
  • Diagnosis
    • Clinical
    • Based on musculoskeletal, ocular, hereditary, and CV features
      • Arm span exceeds height and upper segment <0.86 of lower segment
      • Body habitus with kyphoscoliosis, pectus abnormalities, hypermobile joints
      • Ectopic lenses leading to severe myopia
      • MVP (90%), ascending aortic aneurysm (80%), and AI
    • Dissection is the major cause of death in these patients
  • Average life expectancy 40s
marfan s syndrome
Marfan’s Syndrome
  • Surgical Therapy
    • Ascending aortic issues
      • Treated with resection and graft repair
    • Descending aorta involvement rare
      • Medical therapy may be equal if end organ ischemia not present
    • Chronic disease (aneurysmal dilatation)
      • Elective replacement at >6cm
      • Beta blocker therapy
ehlers danlos syndrome
Ehlers-Danlos Syndrome
  • First described in 1682
  • Incidence 1:150,000 people, M=F
  • Clinical characteristics
    • Joint hypermobility
    • Increased skin elasticity
    • Easy bruising
    • Abnormal scarring
      • Wide splitting and gross ecchymoses “cigarette paper scars”
  • 12 different types based on clinical presentation, genetics, and biochemical defects
  • Autosomal dominant with incomplete penetrance
    • only 50% of cases have family history
    • Genetic defects lead to abnormal structure, synthesis, or secretion of type III collagen
      • Leads to weakness of vessel walls with ruptures, dissections, or aneurysm formation
type iv eds
Type IV EDS
  • Arterial defects rare and occur primarily in Type IV (4% of patients)
      • Do not present with classic symptoms
  • Clinical
    • Spontaneous colon perforations, splenic ruptures, uterine ruptures, PTX
    • CVAs common
    • Most patients unaware of disease until affected by catastrophic event
      • Ruptured artery, dissection, aneurysm formation in patient in late 20s
        • Can occur spontaneously or with minor trauma
      • Cikrit et al JVS 5:248, 1987
        • Femoral-popliteal 27%
        • Aortoiliac 25%
        • Carotid, vertebral, subclavian, axillary 23%
        • Visceral 17%
        • Brachial/radial 6%
  • Invasive diagnostic tests or monitoring should not be used
    • Large retroperitoneal hematomas simply from femoral venous catheters
    • Arteriography has 67% complication rate including death
    • Should use duplex, CT-A, MRA
management of eds
Management of EDS
  • Expectant until vascular event occurs
    • If bleeding is not active and limb is viable then conservative treatment with bedrest
    • Operative intervention should be avoided unless clearly necessary
      • Simple exposure of vessels can be problematic
      • Massive hemorrhage can occur due to friability
      • Simple ligation with umbilical tape may be best option
      • Bypasses can be reinforced with teflon pledgets
        • Fibrin glue
  • High mortality with 44% of patients dying before intervention and 20% after
    • No cure
    • 90% of patients die before middle age from catastrophic vascular event
persistent sciatic artery
Persistent Sciatic Artery
  • Fewer than 100 reported cases
    • Prevalence of 0.25 per 1000 patients studied by angiography
  • Embryology
    • Femoral plexus
      • Supplied ventrally by hypogastric to evolve later into fem-pop system
      • Supplied dorsally by axial artery to later regress to gluteal artery
    • Complete form of syndrome the sciatic vessel communicates directly with popliteal artery
      • Creates paradox where femoral pulse is absent but distal pulses full “Cowie’s sign”
slide19
Clinical Findings
    • Diagnosed in patients around 50 yo but may be noted throughout life
    • M=F
    • Bilateral in 1/3
    • Usually found incidentally on arteriography or autopsy
    • When symptomatic will present with aneurysm development in pelvic portion of artery
      • Pulsatile buttock mass
      • Distal embolization
      • Rupture
      • Compression of sciatic nerve
  • Treatment
    • Surgery indicated for rupture, symptomatic aneurysms, ischemic complications
    • Ligation and embolization are effective
uncommon arteriopathies20
Uncommon Arteriopathies
  • Vasculitis
    • Temporal arteritis
    • Periarteritis Nodosa
    • SLE
    • Behcet’s disease
    • Kawasaki’s disease
    • Rheumatoid disease with vasculitis
    • Relapsing polychondritis
  • Congenital diseases with arterial involvement
    • Marfan syndrome
    • Ehlers-Danlos syndrome
    • Pseudoxanthoma elasticum
    • Homocystinurea
    • Neurofibromatosis
    • Tuberous sclerosis
    • Coarctation of the abdominal aorta
    • Persistent sciatic artery
  • Uncommon acquired arteriopathies
    • Tumor
    • Radiation
    • Focal calcific aortic obstruction
    • Iliac syndrome in cyclists
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