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    Presenter

    Dr. Omar Sadeque KhanMS 3rd part Student

    Moderator

    Dr. Md. Enamul HakimAssistant Professor

    Vascular Surgery

    NICVD

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    TTakayasus arteritis (TA) is a disease of unknown etiology with a

    constellation of clinical findings primarily resulting from effects on the

    aorta and its branches.

    Various names used to describe the condition have been based on

    associated clinical findings: middle aortic syndrome, pulseless

    disease, young female arteritis, nonspecific aortoarteritis, and aortic

    arch syndrome.

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    TTA is an uncommon disease, although its incidence depends highly on

    geography. In the United States, an accepted figure is 2.6 cases per 1 million

    people, whereas Japanese autopsy data reflects a number of 1 case per 3000

    people.

    It has a world wide distribution but is more common in young Asian womenwith typical onset at the age of 25-30 yrs .

    Female : male ratio 8:1.

    IIn the Western world, atherosclerosis is a more frequent cause of obstruction

    of the aortic arch vessels than is Takayasu's arteritis.

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    TTakayasus arteritis is named in honor of Dr. Mikito Takayasu, a

    Japanese ophthalmologist who in 1908 presented his findings of

    arterio-venous changes of the ocular papilla in a 21-year-old woman.

    Interestingly, two other physicians at the same meeting presented

    similar findings, noting also the absence of radial pulses in their

    patients.

    Yasuzo Shinmi gave credit to Takayasu and used the name

    Takayasus arteritis in describing a patient in 1939.

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    Original Criteria for Takayasus Arteritis

    CRITERIA DEFINITION

    All patients < 40 years old Age < 40 years at diagnosis or at onset of symptoms

    Two major criteria:

    (1) Left midsubclavian artery lesion Severe stenosis or occlusion in midportion from 1 cm proximal to the

    vertebral artery origin to a point 3 cm distal to the origin

    (2) Right midsubclavian artery lesion Severe stenosis or occlusion in the midportion from right vertebral arteryorigin to a point 3 cm distal to the origin

    Nine minor criteria:

    (1) Elevated erythrocyte sedimentation

    rate (ESR)

    Unexplained ESR > 20 mm/hr

    (2) Carotid tenderness Unilateral or bilateral tenderness of common carotid artery on palpation;

    differentiate from muscle tenderness

    (3) Hypertension Persistent elevation of blood pressure, 140/90 mm Hg (brachial) or 160/90mm Hg (popliteal)

    (4) Aortic regurgitation/annular disease By auscultation, echocardiography, or angiography

    (5) Pulmonary artery disease Lobar or segmental artery occlusion or equivalent; stenosis, aneurysm,

    luminal irregularity in pulmonary trunk or pulmonary arteries

    (6) Left midcommon carotid lesion Severe stenosis or occlusion in midportion 5 cm in length from a point 2

    cm distal to its origin

    (7) Distal brachiocephalic trunk lesion Involves distal third

    (8) Descending aortic lesion Narrowing, dilatation or aneurysm, luminal irregularity, or combination of

    these findings involving thoracic aorta

    (9) Abdominal aortic lesion Narrowing, dilatation or aneurysm, luminal irregularity, or combination of

    these findings involving abdominal aorta plus the absence of distal aortic

    (last 2 cm) and iliac disease

    Adapted from Ishikawa K: Diagnostic approach and proposed criteria for the clinical diagnosis of Takayasus arteriopathy. J Am

    Coll Cardiol 12:964972, 1988; and Vanoli M, Bacchiani G, Origg L, Scorza R: Takayasus arteritis: A changing disease. J

    Nephrol 14:497505, 2001.

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    Sharmas Criteria for Takayasus Arteritis *

    CRITERION DEFINITION

    Three major criteria:

    (1) Left midsubclavian artery

    lesion

    Severe stenosis or occlusion in midportion from 1 cm proximal to the

    vertebral artery origin to a point 3 cm distal to the origin

    (2) Right midsubclavian artery

    lesion

    Severe stenosis or occlusion in the midportion from right vertebral artery

    origin to a point 3 cm distal to the origin

    (3) Characteristic signs and

    symptoms of at least 1 months

    duration

    May include the following: claudication, decrease/absence of pulses, > 10

    mm Hg blood pressure (BP) differences between limbs, fever, neck pain,

    amaurosis fugax, blurred vision, syncope, dyspnea, palpitations

    Ten minor criteria:

    (1) Elevated erythrocyte

    sedimentation

    Unexplained ESR > 20 mm/hr rate (ESR)

    (2) Carotid tenderness Unilateral or bilateral tenderness of common carotid artery on palpation;

    differentiate from muscle tenderness

    (3) Hypertension Persistent elevation of BP, 140/90 mm Hg (brachial) or 160/90 mm Hg

    (popliteal)(4) Aortic regurgitation/annular

    disease

    By auscultation, echocardiography, or angiography

    (5) Pulmonary artery disease Lobar or segmental artery occlusion or equivalent; stenosis, aneurysm,

    luminal irregularity in pulmonary trunk or pulmonary arteries

    (6) Left midcommon carotid

    lesion

    Severe stenosis or occlusion in midportion 5 cm in length from a point 2

    cm distal to its origin

    (7) Distal brachiocephalic trunk

    lesion

    Involves distal third

    (8) Descending aortic lesion Narrowing, dilatation or aneurysm, luminal irregularity, or combination of

    these findings involving thoracic aorta

    (9) Abdominal aortic lesion Narrowing, dilatation or aneurysm, luminal irregularity, or combination of

    these findings involving abdominal aorta

    (10) Coronary artery lesion Angiographically documented in patient < 30 years old and without risk

    factors such as hyperlipidemia and diabetes mellitus

    Adapted from Sharma BK, Iliskovic NS, Singal PK: Takayasus arteritis may be underdiagnosed in North

    America. Can J Cardiol 11:311316, 1995; and Vanoli M, Bacchiani G, Origg L, Scorza R: Takayasus arteritis: A

    changing disease. J Nephrol 14:497505, 2001.* Diagnosis of TA requires two major elements orone major and two minor elements orfour minor elements.

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    American College of Rheumatology Criteria forTakayasus Arteritis (TA) *

    CRITERIA DEFINITION

    Age at disease onset in years Symptoms/findings of TA at < 40 years

    Claudication Lower or upper extremity muscle fatigue during exercise

    Decreased brachial artery pulse Unilateral or bilateral

    Blood pressure (BP) difference

    > 10 mm HgMeasured systolic BP between upper extremities

    Bruit over subclavian arteries or

    aortaOne or both subclavians or abdominal aorta

    Angiographic abnormalities

    Narrowing or occlusion of aorta, primary branches, or the large arteries in

    proximal extremities; must not be secondary to arteriosclerosis,

    fibromuscular dysplasia, or other causes; usually focal or segmental

    Adapted from Arend WP, Michel BA, Bloch DA: The American College of Rheumatology 1990 criteria for the

    classification of Takayasus arteritis. Arthritis Rheum 33:11291134, 1990; and Vanoli M, Bacchiani G,

    Origg L, Scorza R: Takayasus arteritis: A changing disease. J Nephrol 14:497505, 2001.

    * If three or more of these criteria are present, Takayasus arteritis can be diagnosed with a specificity of

    97.8% and a sensitivity of 90.5%.

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    TThecause and pathogenesis are unknown, Although autoimmune

    mechanisms are suspected. HLA haplotype A24-B52-DR2 has beenfound in Asian populations.

    GGrossly, it produces irregular thickening of the aortic wall with

    intimal wrinkling.

    MMicroscopically, early stages show adventitial perivascular(vasa vasorum) mononuclear cell infiltrate, followed in later stages

    by medial fibrosis, occasionally with granulomas and acellular

    intimal thickening.

    OOne explanation for the formation of aneurysms in TA is that the

    destructive process in the media occurs too rapidly, before fibrosisof smooth muscle can develop.

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    SIZE OF THE VESSELS INVOLVEMENT IN

    VASCULITIS

    Large Vessel

    Giant cell arteritis Takayasus arteritis

    Medium vessel

    Classical polyarteritis

    nodosa

    Kawasaki disease

    Small vesselMicroscopic polyangiitis

    Wegners granulomatosis

    Churg-Strauss Syndrome

    Henoch-Schnlein purpura

    Mixed essential

    cryoglobulinaemia

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    CClaudication and systemic symptoms of fever, arthralgia andweight loss, ocular disturbances including visual defects,

    retinal hemorrhages and total blindness.

    OO/E: loss of pulses, bruits, hypertension.

    CCan have numerous cardiac sequelae, including directcoronary involvement and aortic insufficiency with resultant

    congestive heart failure.

    CCoronary arteritis is not common but, when present, tends to

    affect the ostia.

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    Comparison of Various Classification Systems for Takayasus Arteritis

    Ueno classification:

    Type I Disease of the aortic arch and its branches

    Type II Disease confined to the descending thoracic and

    abdominal aorta

    Type III Combination of types I and II

    Type IV Any of the above features with pulmonary artery

    involvement (Lupi-Herrera modification)

    Nasu classification:

    Type I Disease limited to vessels originating from the

    aortic arch

    Type II Also involves aortic root and arch

    Type III Localized to subdiaphragmatic aorta

    Type IV Entire aorta and its branches involved

    Tokyo International Conference on Takayasus Arteritis classification*

    :

    Type I Aortic arch branches alone

    Type IIa Ascending aorta, arch and branches

    Type IIb IIa plus descending thoracic aorta

    Type III Descending thoracic aorta and abdominal

    aorta/branches

    Type IV Abdominal aorta/branchesType V Entire aorta and branches

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    INVESTIGATIONS

    Laboratory investigation are usually non-specific.

    ESR- Raised.

    PBF- Normocytic Normochromic Anaemia.

    ANCA- c- ANCA/ p- ANCA- Positive. Conventional Angiography- Coarctation / Occlusion /

    Aneurysmal dilatation.

    MRA or CT angiogram.

    Some of the newer measurements available, such as of IL-6and RANTES, have not yet been studied conclusively in the

    clinical realm.

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    TREATMENT

    Medical management Steroids are effective in treating the acute inflammation of

    TA. ( High-Dose Oral Prednisolone 1-2 mg/ kg daily)

    Dosage was tapered using the ESR as an index of

    disease activity .Most patients continued their treatment forseveral years, staying on doses as high as 15 mg/day.

    Cytotoxic drugs used for TA include cyclophosphamide,

    methotrexate, and azathioprine.

    Methotrexate may be especially effective and may have manyfewer side effects than the other agents.

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    TREATMENT CONT..

    When considering medical therapy, both the clinician and

    the patient must recognize the limitations of treatment but also

    realize that some patients attain stable remission with therapy.

    Indeed, flareup is unusual if remission lasting 5 years can be

    attained.

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    TREATMENT CONT..

    Surgery-

    Open surgical intervention has been the mainstay of

    treatment for many years, but catheter-based procedures

    are finding greater application.

    Angioplasty and stenting has a high rate of recurrent

    symptoms and restenosis.

    Bypass remains the standard operative approach to most TA

    lesions.

    Endarterectomy is a poor choice because of the extensive

    inflammation and transmural nature of the disease process.

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    Indications for surgery

    Cerebrovascular disease due to the presence of complete occlusivelesions of carotid or innominate arteries.

    This treatment usually takes the form of a bypass graft from theascending aorta to an uninvolved distal target site. The ascendingaorta is chosen because of the relative infrequency with which it isinvolved in the disease process. A 10-mm synthetic graft can be

    sewn after the placement of a side-biting clamp, and additionallimbs can be added if necessary for bypass to other vessels.

    Renal stenosis and renal artery hypertension in patients with TAhave traditionally been treated with vein bypass .

    Clinically significant aortic regurgitation may occur in up to

    44% of patients with TA, and aortic valve repair orreplacement is being performed more commonly for thiscondition.

    Aneurysmal dilatation.

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    PROGNOSIS

    The 5-year survival rate is ~80%.

    Patients older than 35 years at the time of surgery had a

    2.74-fold higher risk of dying than younger patients.

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    K Ci J 2009 D 39(12) 551 5 E b 2009 D 30

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    Korean Circ J. 2009 Dec;39(12):551-5. Epub 2009 Dec 30.

    Takayasu's Arteritis Involving the Ostia of Three Large Coronary

    Arteries.Park JS, Lee HC, Lee SK, Kim SP, Kim YD, Ahn MS, Hong TJ.Department of Internal Medicine/Cardiac Catheterization Laboratory, Pusan National University

    Hospital, Busan, Korea.Takayasu's arteritis can involve the ostia of coronary arteries. We report a patient with

    Takayasu's arteritis involving the ostia of three large coronary arteries who was

    successfully treated by percutaneous coronary intervention (PCI) with a drug-eluting

    stent (DES) and had a good clinical outcome after 12 months. A 37-year-old male

    with unstable angina was admitted to our cardiovascular center. The patient had

    Takayasu's arteritis and an aortic valve replacement with a metallic valve due tosevere aortic regurgitation 7 years previously. Coronary angiography (CAG) showed

    a 95% discrete eccentric luminal narrowing at the ostia coronary artery (RCA). The

    patof the large left anterior descending (LAD) and left circumflex (LCX) arteries, and

    a 99% discrete eccentric luminal narrowing at the ostium of the large right ient was

    treated with prednisolone for 14 days. Two large paclitaxel-eluting stents (PES) were

    then implanted in the distal left main coronary artery using the kissing stent

    technique. After 6 months, a CAG did not reveal restenosis or recurrent coronary

    artery disease. Thus, PCI with a DES for patients with significant coronary

    involvement secondary to Takayasu's arteritis is an effective and an alternative

    treatment when coronary bypass grafting is not option.

    Research Article

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    Research Article

    Lack of Antilipoprotein Lipase Antibodies in TakayasusArteritisJozelio Freire de Carvalho,1 Rosa Maria Rodrigues Pereira,1 Vilma Santos Trindade Viana,1

    Elosa Bonf a,1 and Yehuda Shoenfeld2

    1Disciplina de Reumatologia, Faculdade de Medicina da Universidade de Sao Paulo, 0124-6903 Sao Paulo, SP, Brazil

    2Department of Medicine B, Center for Autoimmune Diseases, Sheba Medical Center, 52621 Tel-Hashomer, Israel

    Correspondence should be addressed to Jozelio Freire de Carvalho, [email protected]

    Received 8 April 2009; Accepted 23 May 2009Recommended by Eiji Matsuura

    Background. Antilipoprotein lipase (anti-LPL) antibodies were described in rheumatic diseases.

    In systemic lupus erythematosus they were highly associated with inflammatory markers and

    dyslipidemia, and may ultimately contribute to vascular damage. The

    relevance of this association in Takayasus arteritis, which is characterized by major

    inflammatory process affecting vessels, has not been determined.

    Objectives. To analyze the presence of anti-LPL antibodies in patients with Takayasus arteritisand its association with inflammatory markers and lipoprotein risk levels.

    Methods. Thirty sera from patients with Takayasus arteritis, according to

    ACR criteria, were consecutively included. IgG anti-LPL was detected by a standard ELISA.

    Lipoprotein risk levels were evaluated according to NCEP/ATPIII. Inflammatory markers

    included ESR and CRP values.

    Results. Takayasus arteritis patients had a mean age of 34 years old and all were females. Half

    of the patients presented high ESR and 60% elevated CRP. Lipoprotein NCEP risk levels were

    observed in approximately half of the patients: 53% for total cholesterol, 43% for triglycerides,

    16% for HDL-c and 47% for LDL-c. In spite of the high frequency of dyslipidemia and

    inflammatory markers in these patients no anti-LPL were detected.

    Conclusions. The lack of anti-LPL antibodies in Takayasus disease implies distinct mechanisms

    underlying dyslipidemia comparedto systemic lupus erythematosus.

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    CONCLUSIONS

    TA has a varied presentation leading to a host of clinical problems for

    which the vascular surgeon may be consulted. Because it is an

    uncommon disorder, many of the treatment decisions must be made

    based on limited information, particularly with regard to the newer

    catheter-based interventions. Both the immediate vascular sequelae

    and the systemic nature of the illness must be taken into account

    before one embarks on treatment. A multidisciplinary approach is

    appropriate, and careful long-term follow-up is necessary for this

    diverse and challenging group of patients.

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    THANK YOU