Uncommon arteriopathies
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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 l.jpg
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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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



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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


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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


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Temporal Arteritis

  • Second most common pattern is bilateral involvement of profunda and SFA

  • Angiography

    • Multiple stenotic areas

    • Post-stenotic dilatation

    • Generous collaterals develop


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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


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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


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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


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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


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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


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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


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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


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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


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    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


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    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”


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    • 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


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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

      • 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