1908 TAKAYASU FIRST CASE REPORT ONISHI PULSELESSNESS 27 OCT 1939 DEPT OF PSYCHIATRY TOKYO DEATH DUE TO HF AUTOPSY PANARTERITIS OF AORTA BL CCA AND LT SCA FROVIG CASE REPORT OF WOMAN WITH BL CAROTID PULSE OBLITERATION ID: 775383
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TAKAYASU ARTERITIS
DR.VIGNESH SUKUMAR
Slide2Slide31908 - TAKAYASU FIRST CASE REPORTONISHI - PULSELESSNESS27 OCT 1939 - DEPT OF PSYCHIATRY, TOKYO - DEATH DUE TO HF – AUTOPSY – PANARTERITIS OF AORTA, B/L CCA AND LT SCAFROVIG - CASE REPORT OF WOMAN WITH B/L CAROTID PULSE OBLITERATION
HISTORY
Slide4ROSS & MCKUSICK- 100 CASES –AORTIC ARCH SYNDROME- YOUNG FEMALE ARTERITIS1963-HIDEO UEDA- MANY CASE STUDIES – AORTA AND MAIN BR, CORONARY & PULMONARY ARTERY – PANAORTITIS AORTITIS SYNDROME – PROBABLE AUTOIMMUNE CAUSE1975- DEPT OF HEALTH & WELFARE, JAPAN – 1ST COINED - TAKAYASU ARTERITIS
HISTORY
Slide5AGE OF ONSET < 40CLAUDICATION OF EXTREMETIESDECREASED BRACHIAL ARTERY PULSEBLOOD PRESSURE DIFFERENCE >10MMHG BETWEEN ARMSBRUIT OVER SUBCLAVIAN ARTERY OR AORTAARTERIOGRAM ABNORMALITY- OCCLUSION OR NARROWING OF AORTA OR MAIN BRANCHES
1990 ACR CLASSIFICATION CRITERIA:- 3/6
Slide6OBLIGATORY CRITERIAAGE <40 AT DIAGNOSIS OR DISEASE ONSETMAJOR CRITERIAINVOLVEMENT OF LEFT SUBCLAVIANINVOLVEMENT OF RIGHT SUBCLAVIANMINOR CRITERIAHIGH ESR, CAROTIDYNIA, HYPERTENSION, AORTIC REGURGITATION OR ANNULO AORTIC ECTASIA, LESIONS OF PULMONARY ARTERY, LEFT MID CCA, DISTAL BRACHIOCEPHALIC TRUNK, THORACIC AORTA AND ABDOMINAL AORTAOBLIGATORY CRITERIA PLUS 2 MAJOR OR ONE MAJOR PLUS 2 OR MORE MINOR, OR 4 OR MORE MINOR
ISHIKAWA CRITERIA
Slide7REMOVAL OF OBLIGATORY AGE CRITERIAADDITION OF CORONARY ARTERY LESION IN ABSENCE OF RISK FACTORSREMOVAL OF AGE IN DEFINING HYPERTENSION (PREV <40YRS)
SHARMA MODIFIED CRITERIA- 1995
Slide8MAJOR CRITERIALEFT MIDSUBCLAVIAN ARTERY LESIONRIGHT MID SUBCLAVIAN ARTERY LESIONCHARACTERISTIC SYMPTOMS AND SIGNS FOR >1MONTHLIMB CLAUDICATIONPULSELESSNESS OR BP DIFF >10MMHG IN ARMSEXERCISE ISCHAEMIANECK PAINFEVERAMAUROSIS FUGAXSYNCOPEDYSPNOEAPALPITATIONSBLURRED VISION
SHARMA MODIFIED CRITERIA
Slide9HIGH ESR >20MM/HR WESTERGREN`SCAROTIDYNIAHYPERTENSION BRACHIAL BP >140/90 POPLITEAL BP >160/90AR OR ANULOAORTIC ECTASIA BY AUSCULTATION OR ECHO OR ARTERIOGRAPHYPA LESION: LOBAR OR SEGMENTAL OCCLUSION OR STENOSIS OR ANEURYSM OF MAIN PTLEFT MIDDLE CCA LESION: STENOSIS OR OCCUSION OF MIDDLE 5 CM STARTING 2CM FROM ORIFICEDISTAL INNOMINATE ART LESION: STENOSIS OR OCCLUSION OF DISTAL 3RDDTA LESION: NARROWING ANEURYSM OR LUMINAL IRREGULARITYABD AORTA LESION: NARROWING ANEURYSM OR LUMINAL IRREGULARITYCORONARY ART LESION : DOCUMENTED BY CAG IN PTS <30YRS AND WITHOUT RISK FACTORS FOR ATHEROSCLEROSIS
MINOR CRITERIA
Slide10CRITERIASENSITIVITYSPECIFICITYISHIKAWA60.495ACR CRITERIA77.495SHARMA ET AL92.595
IAS- 60,80,90
Slide11PRE PULSELESS PHASE OF DISEASECONSTITUTIONAL SYMPTOMS – FEVER MYALGIA, WEIGHT LOSS, ARTHRALGIA, HEADACHEPULSELESS PHASE OR LATE OCCLUSIVE PHASEVASCULAR INSUFFICIENCYDIMINISHED OR ABSENT PULSESBRUITSHYPERTENSIONRENAL ARTERY STENOSISAORTIC REGURGITATION AND HEART FAILURE
TWO STAGE PROCESS
Slide12USUALLY CAUSES WALL EROSION AND FORMATION OF FALSE OR TRUE ANEURYSMSCOMBINATION OF STENOSIS AND ANEURYSM IS RARE IN TB ARTERITISPREDOMINANTLY INVOLVES DESC THO AORTA AND ABDOMINAL AORTA. ASCENDING AORTA IS USUALLY SPARED IN TB ARTERITISRENAL ARTERY INVOLVEMENT IS RAREDISSECTION AND RUPTURE OF ANEURYSMS ARE MC IN TB ARTERITS WHICH IS DISTINCTLY UNCOMMON IN TAKAYASU`S
TB ARTERITIS ???
Slide13LUPI HERERA ET AL48% OF JAPANESE PTS WERE FOUND TO HAVE TUBERCULOSISSEN ET AL71% OF INDIAN PTS WERE FOUND TO HAVE CO EXISTING TUBERCULOSISSUBRAMANYAN ET AL (SCTIMST)(16/88)18.2% HAD SKIN TEST +VE AND WERE GVEN ATTONLY 3 HAD ACTIVE PULMONARY DISEASE
Slide14PRESENTS AT AN OLDER AGEOFTEN INVOLVES ASCENDING AORTADTA IS SPAREDSTENOSIS OF AORTA AND LARGE ARTERIES ARE NOT A FEATURE OF LEUTIC AORTITS
LEUTIC AORTITIS ???
Slide15TYPESARTERIAL INVOLVEMENT1BRANCHES OF AORTIC ARCH2AASCENDING AORTA, AORTIC ARCH AND BRANCHES2BASCENDING AORTA, AORTIC ARCH AND BRANCHES, AND DESCENDING THORACIC AORTA3DESCENDING THORACIC AORTA ABDOMINAL AORTA AND RENAL ARTERIES4ONLY ABDOMINAL AORTA AND RENAL ARTERIES5COMBINED 2B AND 4
ANGIOGRAPHIC CLASSIFICATION OF TA
TAKAYASU CONFERENCE 1994
Slide16Slide171.AORTIC ARCH VARIETYANY 1 OF THE 3 INVOLVED2.THORACO ABDOMINAL VARIETYDTA AND AA OR ITS BRANCHES3.COMBINED VARIETYBOTH ARCH AND THORACOABDOMINAL VARIETY4.PULMONARY VARIETYPULMONARY ARTERY INVOLVEMENT IN COMBINATION WITH ANY OF THE THREEPANJA ET AL5. CORONARY ARTERY INVOLVEMENT
LUPI HERRERA CLASSIFICATION
Slide18OCCURS IN <10%OFTEN FATAL3 TYPES1.STENOSIS OR OCCUSION OF OSTIUM2.DIFFUSE OR FOCAL ARTERITIS3.CORONARY ANEURYSMMC IS TYPE 1 – OSTIAL INVOLVEMENT
CORONARY INVOLVEMENT IN TA
Slide19~70% INVOLVEMENT BY ANGIOGRAPHIC STUDIES( ~36% PANJA ET AL INDIA)SEGMENTAL AND SUBSEGMENTAL BRANCHESMORE IN UPPER LOBEHEMOPTYSISNON ANGINAL CHEST PAINDISPROPORTIONATE PAHOLIGEMIC LUNG FIELDS IN CXR ABNORMAL VENTILATION PERFUSION SCAN
PULMONARY ARTERY INVOLVEMENT
Slide20~96% FEMALESAORTIC ARCH AND BRANCHES ARE INVOLVED MORE OFTEN (TYPE 1 AND 2A)MORE AORTIC REGURGITATIONPRESENTATION - UL CLAUDICATION AND PULSELESSNESSVASCULITIS INVOLVES ASC-TA AND DSC-TA FORMS MORE COMPLICATED LESIONS AND PROLONGED INFLAMATORY ACTIVITYHLA B52 AND HLA B29
JAPANESE - TA
Slide21ONLY 63% FEMALESABDOMINAL AORTA AND RENAL ARTERY INVOLVEMENT COMMON (TYPE 4)MC PRESENTATION IS HYPERTENSIONVASCULITIS INVOLVES ABD AORTA AND LOWER INFLAMATORY ACTIVITY LESS COMPLICATED LESIONS AND EXTENSION TO THORACIC AORTA AFTER ONE OR TWO DECADES
INDIAN - TA
Slide22Slide23STAGECLINICAL FEATURESSTAGE 1UNCOMPICATED DISEASESTAGE 2AMILD/ MODERATE SINGLE COMPLICATIONSTAGE 2BSEVERE SINGLE COMPLICATIONSTAGE 3TWO OR MORE COMPLICATIONS
ISHIKAWA CLINICAL CLASSIFICATION OF TA
NORMAL OR ELEVATED ESR WITH OR WITHOUT PULMONARY INVOLVEMENT
4 COMPLICATIONS
RETINOPATHY, SECONDARY HTN, AR AND ANEURYSM FORMATION
Slide24Slide25ISHIKAWA ET AL SURVIVAL AT 5 YEARS AFTER DIAGNOSIS 83.1%CARDIAC FAILURE - MCC OF DEATHCHILDHOOD ONSET WORSE PROGNOSIS ESP WHEN A/W DCMA FAILED ANGIOPLASTY IMPLICATES HIGH MORTALITY
NATURAL HISTORY
Slide2688 PTS - MEAN AGE ~24YRS38.6% WERE MEN (HIGHER)AT 10 YRS OVERALL SURVIVAL – 80% EVENT FREE SURVIVAL - 61%GRPS 1 AN 2A HIGHER EVENT FREE SURVIVAL RATE THAN GRPS 2B AND 3PREDICTORS OF DEATH OR MAJOR EVENTSEVERE HYPERTENSION, SEVERE FUNCTIONAL DISABILITY, CARDIAC INOLVEMENT(CARDIOMEGALY, LVH IN ECG AND LV DYSFUNCTION )
SUBRAMANIYAM ET AL
SRI CHITRA
Slide27ANATOMIC TYPE OF ARTERITIS AND PATHOLOGIC TYPE OF LESION STENOTIC OR ANEURYSMAL DID NOT AFFECT THE EVENT FREE SURVIVAL
Slide28Slide29MC MANIFESTATION IS TORTUOSITY AND DILATION OF RETINAL VEINS WITH SLOW BLOOD CIRCULATIONFFA- DELAYED FILLING OF CHOROIDAL AND RETINAL CIRCULATIONSHYPOPERFUSIVEISCHEMIC OCULAR SYNDROMEAION - CRAOHYPERTENSIVEEXUDATIVE RETINOPATHYRETINAL DETATCHMENT PAPILLOEDEMA
OCULAR MANIFESTATIONS
Slide30HYPERTENSIVE RETINOPATHYKEITH WAGNER CLASSIFICATIONARTERIOLAR NARROWING, AV CROSSING CHANGES, COPPER WIRING, EXUDATES, SILVER WIRING AND PAPILLOEDEMANON HYPERTENSIVE / ISCHAEMIC RETINOPATHY CLASSIFICATIONUYAMA AND ASAYAMA
OCULAR MANIFESTATIONS
Slide31STAGE 1 – DILATION OF SMALL VESSELSSTAGE 2 – MICROANEURYSM FORMATIONSTAGE 3 – WREATH LIKE ARTERIO VENOUS ANASTAMOSIS SURROUNDING OPTIC PAPILLAESTAGE 4 – CATARACT, SECONDARY GLAUCOMA, NEOVASCULARISATION, PROLIFERATIVE RETINOPATHY, VITREOUS HMGMILD : STAGE 1MODERATE: STAGE 2SEVERE: STAGE 3 AND 4
UYAMA AND ASAYAMA CLASSIFIACTION OF RETINOPATHY
Slide32CASE REPORT
CMC VELLORE
Slide33RENO VASCULAR HTNAORTIC REGURGITATIONCAROTID ARTERY STENOSIS (DECREASED PERFUSION TO BARORECEPTORS)THICKENED AND STIFF AORTA
CAUSES OF HYPERTENSION IN TA
GB PANTH, NEW DELHI
Slide34CMC VELLORE
Slide35AGE<16YRS - NOT AS FREQUENT AS ADULTSMORE SEVERE MANIFESTATIONSCAN BE A/W MYOCARDITIS AND DCM LIKE PRESENATIONPRESENTING FEATURES – FEVER, HEADACHE, HTN AND HFAN ASOCIATION WITH TUBERCULOSIS POSTULATED BUT NEVER PROVENHIGH RELAPSE RATE - NEEDS AGGRESSIVE IMMUNOSUPPRESSION
CHILDHOOD TAKAYASU [c-TA]
Slide36PREGNANCY DOES NOT EXACERBATE DISEASEHTN MANAGEMENT IS ESSENTIALMATERNAL COMPLICATIONSPRE ECLAMPSIA, ECLAMPSIA, CHF, PROGRESSIVE RENAL IMPAIRMENTABDOMINAL AORTIC INVOLVEMENT AND DELAY IN SEEKING MEDICAL ATTENTION PREDICTED POOR PERINATAL OUTCOME
PREGNANCY AND TAKAYASU
Slide37OFTEN BILATERAL & OSTIALBEST TREATED BY PTBAGB PANTH STUDYSUCCESS RATE – 89.3%CHILDREN HAD HIGHER PROPORTION OF RESTENOSISSTENT PLACEMENT FOLLOWING PTBAOSTIAL LESIONSLONG SEGMENT LESIONSINCOMPLETE RELIEF OF STENOSES DISSECTION
RENAL ARTERY INVOLVEMENT
Slide38ACTIVE DISEASE ???
YES OR NO
Slide39SYSTEMIC FEATURESFEVER, MUSCULOSKELETAL AND CONSTITUTIONAL SYMPTOMSELEVATED ESRFEATURES OF VASCULAR ISCHAEMIA OR INFLAMMATIONCLAUDICATION, VASCULAR PAIN OR CAROTIDYNIA, DIMINISHED OR ABSENT PULSE, VASCULAR BRUIT, ASSYMMETRIC BP IN BOTH UPPER LIMBSTYPICAL ANGIOGRAPHIC FEATURESNEW ONSET OR WORSENING OF 2 OR MORE FEATURES INDICATE ACTIVE DISEASE
KERR CRITERIA
Slide40DISEASE EXTENT INDEX FOR TAKAYASU ARTERITIS -2005INDIAN TAKAYASU ACTIVITY SCOREITAS - 2010ITAS – 2010 – AESR CRP0 - <20 0 - <51 - 20-39 1 – 6-102 - 40-59 2 – 11-203 - >60 3 - >20
DEI-TAK & ITAS
Slide41Slide42BOTH DEI-TAK AND ITAS DERIVED FROM BVASBIRMINGHAM VASCULITIS ACTIVITY SCALE
Slide43Slide44RECENT STUDIESABSENCE OF CONSTITUTIONAL SYMPTOMS AND NORMAL ESR AND CRP DOES NOT RULE OUT ACTIVE DISEASEPREFER IMAGING MODALITIES
Slide45SEROLOGICAL MARKERS
ARE THEY RELIABLE ???
Slide46In conclusion, the present results suggest that
monitoring of circulating levels of MMP-2 as a helpful marker in diagnosing TA and those of MMP-3 and MMP-9 as disease activity markers might help provide adequate evaluation of treatment and guide therapeutic decision making for individual patients with TA. These measurements can be part of routine hospital laboratory examinations that are easy to perform at low cost. Furthermore, the noninvasive nature of such measurements is attractive, because patients can be spared from invasive angiographic examination.
Matrix
Metalloproteinases
as Novel Disease Markers in
Takayasu
Arteritis
Slide47Slide48MMP-9 PENTRAXIN-3RANTESIL-6MMP 2 IN DIAGNOSING MMP 3 AND 9 IN ASSESSING ACTIVITY
MARKERS OF ACTIVE DISEASE
NOT AFFECTED BY PREDNISOLONE
Slide49Slide50Slide51Slide52Slide53Slide54Slide55Slide56Slide57AORTIC WALL THICKNESS OF
>4MM-
90% SENSITIVITY IN IDENTIFYIN THE INVOLVED ARTERIAL SEGMENT
ACTIVE DISEASE:
INCREASED VESSEL WALL THICKNESS, WALL EDEMA AND MURAL CONTRAST ENHANCEMENT
Slide58Slide59TURKISH STUDY- 2013
UPTAKE ON PET - NOT SPECIFIC
DIFF FROM ATHEROSCLEROSIS DIFFICULT
Slide60GRADE O - LOW GRADE UPTAKEGRADE 1 – UPTAKE PRESENT BUT LOWER THAN LIVERGRADE 2 – UPTAKE SIMILAR TO LIVERGRADE 3 – UPTAKE GREATER THAN LIVER BUT LESSER THAN OR SIMILAR TO CEREBRAL CORTEXGRADE 2 OR MORE IS S/O ACTIVE DISEASE IN TAKAYASU`S
SUV- STANDARDIZED UPTAKE VALUE
Slide61Slide62Slide63REMISSION AND SUSTAINED REMISSION
REMISSION
ABSENCE OF SYMPTOMS
NORMAL INFLAMMATORY MARKERS
NO NEW IMAGING FINDINGS
SUSTAINED REMISSION
REMISSION FOR ATLEAST 6 MONTHS
ON STEROIDS<10MG/DAY
Slide64ANTI TNF AGENTSETANERCEPTINFLIXIMABANTI CD20RITUXIMABIL-6 ANTAGONISTTOCILZUMAB
BIOLOGICS
Slide65FRENCH STUDY
Slide66Slide67Slide68Slide69TURKISH TA PTS HAD AN ASSO WITH HLA B52ESR AND CRP- NO CORRELATION WITH ACTIVITYUSE OF ANTIPLT AGENTS WAS A/W LOWER FREQ OF ISCHEMIC EVENTSIL-6 LEVELS CORRELATED WITH DISEASE ACTIVITY TOCILIZUMAB(IL-6 RECEPTOR ANTAGONIST) HAD PROMISING RESULTS IN REFRACTORY CASESSTENT GRAFTS WERE BETTER THAN UNCOVERED METAL STENTS OR BALOON ANGIOPLASTY
STUDY FROM TURKEY 2013
Slide70ENDOVASCULAR INTERVENTIONSUBCLAVIAN ANGIOPLASTYAORTOPLASTYRENAL ANGIOPLASTYCAROTID ANGIOPLASTYCORONARY ANGIOPLASTYMESENTRIC ANGIOPLASTYPULMONARY ATERY ANGIOPLASTY
INTERVENTIONS
Slide71ARTERIAL LUMEN <30% OF RESIDUAL STENOSISARTERIAL LUMEN ATLEAST 50% LARGER THAN PRE TREATMENT DIAMETERGRADIENT <20MMHG OR DECREASED ATLEAST 15MMHG FROM PRE TREATMENT GRADIENT
A SUCCESSFUL ANGIOPASTY
GB PANTH, DELHI
Slide72~20% REQUIRE SURGERYAORTOCERVICAL BYPASSCERVICO SUBCLAVIAN BYPASSAORTO CORONARY BYPASSAORTO AORTIC BYPASSRENAL ARTERY BYPASSAORTIC ROOT REPLACEMENTNEPHRECTOMY FOR NON-FUNC KIDNEY TO CONTROL HTNCABG LESS PREFERRED
SURGERY
Slide73NEWER STUDIES
CMC VELLORE
Slide74Slide75Slide76Slide77(A and C) Baseline angiograms showing diseased arterial segments containing tangles of corkscrew channels located within the expected confines of the vessel wall In the distal right and proximal left common carotid arteries (indicated by black arrows and white arrows, respectively, throughout panels A to F). (B and D) Corresponding images immediately after angioplasty and stenting show replacement of the manifold channels by a single large conduit
Slide78(E) Baseline aortic arch angiogram showing the diseased common carotid artery segments and bilaterally occluded subclavian arteries (hollow block arrows). (F) Two-year post-intervention follow-up aortic arch angiogram showing patency of carotid and subclavian stents bilaterally.
Slide79THANK YOU