Uncommon Arteriopathies

1. 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

2. 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

3. Giant Cell Arteritis

4. 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

5. 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

6. Temporal Arteritis • Second most common pattern is bilateral involvement of profunda and SFA • Angiography • Multiple stenotic areas • Post-stenotic dilatation • Generous collaterals develop

7. 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

8. 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

9. 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

10. 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

11. 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

13. 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

14. 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

15. 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

16. 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

17. 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”

19. 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

20. 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