management of takayasu diseases ,nicvd
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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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