TAKAYASU’S ARTERITIS презентация

TAKAYASU’S ARTERITIS Prepared by: Nurmagambetov Sh. 462 GM

EPIDEMIOLOGY More case reports from Japan ,India, South-east Asia, Mexico No geographic restriction No race – immune Incidence-2.6/million/year-N.America/Europe The incidence in Asia is 1 case/1000-5000 women.

Age Age Mc-2nd & 3rd decade May range from infancy to middle age Indian studies-age 3- 50 yrs Gender diff Japan-F:M=8-9:1 India-F:M ratio varies from -1:1 - 3:1 ( Padmavati S, Aurora AP, Kasliwal RR Aortoarteritis in India. J Assoc Physicians India 1987) India=F:M- 6.4:1 (Panja et al, 1997 JACC)

Genetics Japan - HLA-B52 and B39 Mexican and Colombian patients - HLA-DRB1*1301 and HLA-DRB1*1602 India- HLA- B 5, -B 21

Histopathology Idiopathic c/c infla arteritis of elastic arteries resulting in occlusive &/ ectatic changes Large vessels, esp, Aorta & its main branches (brachiocephalic, carotid, SCL, vertebral, RA) +Coronary & PA Ao valve –usually not beyond IMA Multiple segs with dis & skipped nl areas or diffuse involvement

Gross Gross

Wall thickening, Fibrosis, Stenosis, & Thrombus formation →end organ ischaemia More a/c inflammation → destroys arterial media → Aneurysm (fibrosis inadequate) Stenotic lesions predominate & tend to be B/L Nearly all pts with aneurysms also have stenoses

Associated pathology-TB (LN)-55% Associated pathology-TB (LN)-55% Erthema multiforme Bazins disease(eryt induratum) churg strauss synd reteroperitoneal fib PAN,UC,CD etc

Clinical features Early pre pulseless/gen manif

Coronary involvement in TA Occurs in 10～30% Often fatal Classified into 3 types Type1:stenosis or occlu of coronary ostia Type2:diffuse or focal coronary arteritis Type3:coronary aneurysm

Occular involvement-Amaurosis fugax, pain behind eye, no real visual loss Hypertensive retinopathy

Ishikawa clinical classification of Takayasu arteritis 1978

Cumulative survival Cumulative survival 5years -91% (event free survival -74.9%) 10 years -84% (event free survival -64%) Single mild complication or no complication 5 year event free survival 97% Single severe or multiple complications 5 year event free survival 59.7% No deaths in groups I and IIA 19.6% mortality in groups IIB and III (CVA,CCF)

1990

1995

nee

Findings of TA on MRI mural thrombi signal alterations within and surrounding inflamed vessels vascular dilation thickened aortic valvular cusps multifocal stenoses concentric thickening of the aortic wall Disadvantages difficulty in visualizing small branch vessels and poor visualization of vascular calcification may falsely accentuate the degree of vascular stenoses (renal & subclavian)

[18F]fluorodeoxyglucose PET for diagnosing Takayasu’s arteritis common [18F]FDG uptake pattern TA early phase - linear and continuous late phase-patchy rather than continuous ,linear shown to identify more affected vascular regions than morphologic imaging with MRI does not provide any information about changes in the wall structure or luminal blood flow sensitivities of 83% and specificity 100% ( Meller Jet al. Value of F-18 FDG hybrid camera PET and MRI in earlyTakayasu aortitis. Eur Radiol 2003) Sensitivity of 92%, specificity of 100% and a diagnostic accuracy of 94% ( Webb M et al. The role of 18F-FDG PET in characterising disease activity in Takayasu arteritis. Eur J Nucl Med Imaging 2004

Treatment of TA 　 ・ 　　

Medical treatment

Steroids → 50% response Methotrexate →further 50% respond 25% with active disease will not respond to current treatments resistant to steroids/ recurrent disease once corticosteroids are tapered cyclophosphamide (1-2 mg/kg/day), azathioprine (1-2mg/kg/day), or methotrexate (0.3 mg/kg/week) Mycophenolate mofetil/ anti TNF α agents- infliximab

Critical issue is in trying to determine whether or not disease is active During Rx- regular clinical examination and ESR+ C-RP initially - every few days CT or MR angio - 3 to 12 months - (active phase of Rx), and annually thereafter Criteria for active disease

chronic phase- persistent inflammation steroids should be continued – <1.0 mg/dL of s.C-RP and 20 mm/h of ESR

Surgical treatment HTN with critical RAS Extremity claudication limiting daily activities Cerebrovascular ischaemia or critical stenoses of ≥3 cerebral vessels Moderate AR Cardiac ischaemia with confirmed coronary involvement Aneurysms Recommended at quiescent state-avoids compli (restenosis, anastamotic failure, thrombosis, haemorrhage, & infection)

Surgical techniques Carry high morbidity & mortality Steno /aneurysm -anastomotic points Progressive nature of TA Diffuse nature of TA

Renal artery involvement Best treated by PTA Stent placement following PTA Ostial lesions Long segment lesions Incomplete relief of stenoses Dissection

Renal PTA - 33 stenoses (20 pts) Renal PTA - 33 stenoses (20 pts) Indi-sev HTN,angio 70% stenosis with pr grad 20mm, nl-ESR Tech success -28 lesions (85%) clin success-14(82%) Failures - Coexistent abd Ao disease & tight, prox RAS Tech diffi - tough, noncompliant stenoses, difficult to cross & resisted repeated, prolonged balloon inflations - backache & ↓SBP during balloon inflation Follow-up –mean (8/12) -restenosis in 6 (21%) Renal PTA in TA -tech difficulties; Short-term results - good, Complication rate-acceptable

Aortoarteritic lesions Balloon dilation safe & reasonably effective Can be performed repeatedly without any added risks Balloon dilation diff from atherosclerotic lesions Minimal intimal involvement –permits easy wiring and balloon crossing Resistance to dilation – high fibrotic element in the stenotic lesion restenosis> frequent in TA - diffuse and long stenotic lesions

Joseph s et al, SCT J Vasc Interv Radiol 1994;5:573–580

Aortoplasty and Stenting PTA -desc thoracic and/or abd Ao (TA) stenosis 16 pts (12+4)- HTN/severe b/l- LL claudication Aortography – stenosis→ DTA-5, abd Ao-10, Both -1 Initial tech & clinical success -100% patency rate of 67% in a 52-month follow-up Follow-up (mean 21months)- Restenosis -3 PTA has a definite role in TA management residual gradient < 20 mm -criterion for successful aortoplasty long-segment disease, dissection or persistence of a grad > 20 mm Hg after PTBA- aortic stenting

Treatment for cor A occulusion in TA Surgery (CABG)- often not indicated ・IMA can’t be used often occlu of Innomi A / Scl A calcification of aorta High incidence of restenosis:36％ Angioplasty(PTCA) ・alternative to surgery Very high incidence of restenosis:78％ DES-effectiveness ?

   Percutaneous Management of Aneurysmal Lesions Aneurysmal dilatation- isolation or together with stenotic lesions fusiform or saccular one of the major complications related to the prognosis in TA Incidence of aneurysm rupture -low Management - mainly surgical. Covered stent-grafts may be useful