TAKAYASU’S TAKAYASU’S ARTERITIS – ARTERITIS –

a typical course of a typical course of an untypical disease- an untypical disease-

a case reporta case reportAnna KapłańskaAnna KapłańskaAndrzej ŁabykAndrzej Łabyk

Sławomir TymińskiSławomir Tymiński

Students’ Research Group,Students’ Research Group,Department of Internal Medicine, Hypertension and Department of Internal Medicine, Hypertension and

Vascular Diseases, Warsaw Medical UniversityVascular Diseases, Warsaw Medical University

BACKGROUNDBACKGROUND

Takayasu arteritis (TA)Takayasu arteritis (TA)

systemic inflammatory disease of systemic inflammatory disease of unknown originunknown origin

affects primarily large vessels affects primarily large vessels including aorta and its branchesincluding aorta and its branches

due to variable clinical due to variable clinical manifestations it is a diagnostic manifestations it is a diagnostic challengechallenge

AIMAIM

To present the variety of To present the variety of symptoms, typical course, symptoms, typical course,

complications( in connection complications( in connection with arterial involvement with arterial involvement

localization) and diagnostic localization) and diagnostic difficulties in patient with TAdifficulties in patient with TA

METHODMETHOD

a case reporta case report 30-year-old woman30-year-old woman follow-up – 1.5 yearfollow-up – 1.5 year type type V P(+)V P(+) of TA of TA

TYPES OF TATYPES OF TAtyptypee

involvement of the arteriesinvolvement of the arteries

II Branches from the aortic archBranches from the aortic arch

IIaIIa Ascending aorta, aortic arch & its branchesAscending aorta, aortic arch & its branches

IIbIIb Ascending aorta, aortic arch, its branches & Ascending aorta, aortic arch, its branches & thoracic descending aortathoracic descending aorta

IIIIII Thoracic descending aorta, abdominal aorta Thoracic descending aorta, abdominal aorta &/or renal arteries&/or renal arteries

IVIV Abdominal aorta &/or renal arteriesAbdominal aorta &/or renal arteries

VV Combine features of types IIb and IVCombine features of types IIb and IV

C(+) coronary arteries involvementP(+) pulmonaty arteries involvement

„New angiografic classification of TA” TA conference, Angiology 1997; 48:369-79

ACR CRITERIA FOR TA ACR CRITERIA FOR TA CLASSIFICATIONCLASSIFICATION

1.1. Age at disease onset <40Age at disease onset <40

2.2. Decreased brachial artery pulse (one or Decreased brachial artery pulse (one or both)both)

3.3. SBP difference >10 mmHg (between arms)SBP difference >10 mmHg (between arms)

4.4. Bruits over SAs / abdominal aortaBruits over SAs / abdominal aorta

5.5. Arteriogram abnormalitiesArteriogram abnormalities

6.6. Claudication of extremitiesClaudication of extremities

TA if present >= 3/6TA if present >= 3/6

Sensitivity 90%, specificity 98%Sensitivity 90%, specificity 98%

RESULTSRESULTSCLINICAL CLINICAL

MANIFESTATIONS (1)MANIFESTATIONS (1)On admission/ present complainsOn admission/ present complains HaemoptysisHaemoptysis Pleuritic chest pain Pleuritic chest pain

main reasons for admissionmain reasons for admission

Chronic, non-productive coughChronic, non-productive cough Large joints artralgia (ankle & knee)Large joints artralgia (ankle & knee) Thoracic spinalgia Thoracic spinalgia Progressive weaknessProgressive weakness

CLINICAL CLINICAL MANIFESTATIONS (2)MANIFESTATIONS (2)

In anamnesisIn anamnesis Syncope ( twice, 2 years ago)Syncope ( twice, 2 years ago) Claudication of R upper extremityClaudication of R upper extremity Heart palpitations (for many yrs)Heart palpitations (for many yrs) TachycardiaTachycardia Pregnancy induced HA Pregnancy induced HA HA after pragnancy ( 240/100 mmHg HA after pragnancy ( 240/100 mmHg

despite treatment)despite treatment) Raised temperature ( for 3 months)Raised temperature ( for 3 months) No PMH of altered visual acuityNo PMH of altered visual acuity

CLINICAL CLINICAL MANIFESTATIONS (3)MANIFESTATIONS (3)

On examinationOn examination MalnutritionMalnutrition PallorPallor Greyish nodules on forearms (similar Greyish nodules on forearms (similar

to erythema nodosum)to erythema nodosum) Absent brachial & radial pulsesAbsent brachial & radial pulses Murmurs over aorta, renal arteries, Murmurs over aorta, renal arteries,

carotids, SAscarotids, SAs No retinal changes on fundoscopyNo retinal changes on fundoscopy

CLINICAL CLINICAL MANIFESTATIONS (4)MANIFESTATIONS (4)

R BP R BP L L

-/--/- mmHg mmHg ? 70/-? 70/- mmHg mmHg

200/70200/70 mmHg mmHg 195/80195/80 mmHgmmHg

LABORATORY TESTS LABORATORY TESTS ABNORMALITIES (1)ABNORMALITIES (1)

ESRESR ↑ ↑ 195195 mm/h (N: 3-15 mm/h) mm/h (N: 3-15 mm/h)CRPCRP 7.37.3 g/dl (N< 10 g/dl) g/dl (N< 10 g/dl)WBCWBC ↑ ↑ 15.015.0 G/l G/l (N: 4-10.0 G/l) (N: 4-10.0 G/l)RBCRBC ↓ ↓ 3.933.93 T/l T/l (N: 4.2-5.5 T/l) (N: 4.2-5.5 T/l)HbgHbg ↓ ↓ 8.6 8.6 g/dlg/dl (N: 12- 14.0 g/dl) (N: 12- 14.0 g/dl)HctHct ↓ ↓ 26.2 26.2 %% (N: 37-47 %) (N: 37-47 %)MCVMCV ↓ ↓ 66.566.5 fl fl (N: 81-99 fl) (N: 81-99 fl)PLTPLT ↑ ↑ 711711 G/l G/l (N: 150-400 G/l) (N: 150-400 G/l)

LABORATORY TESTS LABORATORY TESTS ABNORMALITIES (2)ABNORMALITIES (2)

Fibrinogen Fibrinogen ↑ ↑ 736 736 mg/dl mg/dl (N: 200-500mg/dl) (N: 200-500mg/dl)IgGIgGIgAIgA in a normal range in a normal rangeIgMIgMIgEIgEUSR USR (-)(-)HIVHIV (-) (-)HbSHbS (-) (-)HCV HCV (-) (-) cANCAcANCA (-)(-)-ive → -ive → (+)(+)-ive ( -ive ( 35.82 U/l) 35.82 U/l)

pANCApANCA (-)(-)-ive → -ive → (+) (+)-ive ( -ive ( 36.38 U/l) 36.38 U/l)

LABORATORY TESTS LABORATORY TESTS ABNORMALITIES (3)ABNORMALITIES (3)

Total protein Total protein 6.6 g/dl6.6 g/dl (N: 6-8.0 g/dl)(N: 6-8.0 g/dl)

AlbuminAlbumin ↓ ↓ 52.4 % 52.4 % (N: 60.3 - 71.4%)(N: 60.3 - 71.4%)

αα-1 globulin ↑ -1 globulin ↑ 4.5 %4.5 % (N: 1.4 -2.9 %)(N: 1.4 -2.9 %)

αα-2 globulin ↑ -2 globulin ↑ 17.7 %17.7 % (N: 7.2 – 11.3 % )(N: 7.2 – 11.3 % )

ββ-globulin ↑ -globulin ↑ 14.3 %14.3 % (N: 8.1 – 12.7 % )(N: 8.1 – 12.7 % )

γγ-globulin -globulin 11.1 %11.1 % (N: 8.7 – 16 % )(N: 8.7 – 16 % )

CHEST CT SCANCHEST CT SCAN

Consolidations in the upper Consolidations in the upper & middle lobe of R lung& middle lobe of R lung

↓↓Lung infarctLung infarct

Angio CT scan (1)Angio CT scan (1)Complete oclussion of R CCA

Sign. stenosis of brachiocephalic trunk

Dilatation of ascending aorta

moderate stenosis of L CCA

Sign. stenosis of L SA

→ strongly suggestive of TA

Angio CT scan (2)Angio CT scan (2)

Aortic aneurysm

Angio CT scan (3)Angio CT scan (3)

Dissecting aneurysm (?)

Abdominal CT scanAbdominal CT scan

narrowing of truncus celiacus

DOPPLER ULTRASOUNDDOPPLER ULTRASOUND

Did Did notnot confirm dissecting aneurysm confirm dissecting aneurysm Confirmed multiple arterial Confirmed multiple arterial

narrowingsnarrowings Revealed Revealed bilateral steal syndromebilateral steal syndrome R R

>> L>> L Renal arteriesRenal arteries - bilaterally double - bilaterally double

but with but with normalnormal blood flow blood flow

ECHOCARDIOGRAPHY ECHOCARDIOGRAPHY (transthoracic)(transthoracic)

Global LV function- normalGlobal LV function- normal No global/focal hipokinesiaNo global/focal hipokinesia Valves’ orifices and gradients- Valves’ orifices and gradients-

normalnormal Dilatatiom of ascending aortaDilatatiom of ascending aorta IAS aneurysmIAS aneurysm

MAIN DIAGNOSTIC & MAIN DIAGNOSTIC & THERAPEUTIC THERAPEUTIC DIFFICULTIESDIFFICULTIES

1.1. General symptoms & signs → the most General symptoms & signs → the most common & the least specific → further common & the least specific → further carreful investigationscarreful investigations

2.2. How and where to detect BP in TA pt?How and where to detect BP in TA pt?

3.3. How much can we reduce BP in TA pt?How much can we reduce BP in TA pt?

4.4. Confusing imaging studies Confusing imaging studies

5.5. PAs involvment → lung infarct → PAs involvment → lung infarct → haemoptysis & chest pain haemoptysis & chest pain

6.6. steal syndrome → impaired cerebral blood steal syndrome → impaired cerebral blood circulation → syncopecirculation → syncope

CONCLUSIONS (1)CONCLUSIONS (1)

1.1. General symptoms of TA may be General symptoms of TA may be similar to other inflammatory similar to other inflammatory diseasesdiseases

2.2. Since there are no specific Since there are no specific laboratory tests for TA, numerous laboratory tests for TA, numerous imaging studies should be imaging studies should be performed to confirm itperformed to confirm it

CONCLUSIONS (2)CONCLUSIONS (2)

33 TA should be considered in pts with TA should be considered in pts with multiple arterial lesions despite its multiple arterial lesions despite its low prevalencelow prevalence

44 Symptoms and complications depend Symptoms and complications depend on the involvement of particular on the involvement of particular arteries and may consist of impaired arteries and may consist of impaired cerebral blood flow, visual problems, cerebral blood flow, visual problems, HA, HP, pulmonary infart HA, HP, pulmonary infart