Duk-Kyung Kim and Young-Wook Kim

A 12-year old Korean girl was presented with neck pain and transiently dimmed vision. One year before presenting, the patient developed fever, malaise and bilateral neck pain followed by right leg claudication. More recently, she experienced dimming of the visual field in both eyes, aggravated when facing upwards. She did not have episodes of imbalance, loss of coordination, diplopia or vertigo. She did not complain of dyspnoea, angina or abdominal angina. Her right arm blood pressure (BP) was 99/54 mmHg but the left arm BP was not checkable. A cardiac examination was normal. Both carotid pulses and the right brachial pulse were weak. The left brachial pulse, right popliteal and right dorsalis pedis pulses were not palpable. Bruit was audible over both carotid arteries and in the supraclavicular, infraclavicular and epigastric area. Neurology disclosed no abnormalities. Basal laboratory examinations revealed a white blood count of 9,700 × 103/mL, erythrocyte sedimentation rate (ESR) 66 mm/h, highsensitivityC-reactiveprotein(hsCRP)1.19mg/dL,protein/albumin7.3/3.8g/dL, creatinine 0.53 mg/dL and pro-brain-type natriuretic peptide (proBNP) 18.3 pg/mL.

Question 1

Which of the patient’s findings does not fulfill diagnostic criteria of Takayasu’s arteritis (TA)?

A.  Age at disease onset <40 years B.  Claudication of extremities C.  Elevated ESR and CRP

D.  Systolic blood pressure (SBP) difference >10 mmHg between arms E.  Bruit over subclavian arteries

Based on her clinical findings, she was diagnosed with Takayasu arteritis.

D.-K. Kim and Y.-W. Kim ( )

Division of Cardiology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea and

Division of Vascular Surgery, Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea

e-mail: dkkim@skku.edu; ywkim@skku.edu

Question 2

The work-up of this patient presenting with TA must include:

A.  Conventional angiography

B.  Duplex ultrasonography of the carotid artery and lower limb arteries C.  Computed tomography (CT) angiography of the aorta

D.  Magneticresonanceimaging(MRI)andmagneticresonance(MR)angiographyofthebrain

Carotid duplex ultrasonography showed diffuse wall thickening (Fig. 35.1) and severe segmental stenosis of both common carotid arteries. There were diffuse 30% stenosis of the right innominate artery, occlusion of the distal portion of the right subclavian artery, 70% stenosis of the proximal portion of the left subclavian artery and total occlusion after the origin of the left vertebral artery. Duplex ultrasonography of the lower extremity arteries revealed long segmental occlusion of the right superficial femoral artery and the right anteriortibialartery.CTangiographyofthethoracoabdominalaortadisclosedwallthickeningof the aortic arch and proximal supratruncal branches, total occlusion of the superior mesenteric artery and well-developed collaterals from the inferior mesenteric artery. Brain MRI demonstrated no findings of acute infarction. MR angiography disclosed further findings with stenosis of proximal portion of the right internal carotid artery (Fig. 35.2).

Question 3

Which of the following statements is false regarding BP of the patient?

A.  The patient’s true BP is 99/54 mmHg.

B.  In patient with TA, BP should be measured in all four extremities.

C.  Renovascularhypertension is themostcommoncauseofhypertensionin patients with TA. D.  Atypical coarctation of the aorta can be a cause of high BP of the upper extremities.

Fig. 35.1  Duplex ultrasonography showing long, smooth, homogenous concentric thickening of the proximal portion of the left common carotid artery. IJV; internal jugular vein, CCA; common carotid artery

Fig. 35.2 Magnetic resonance (MR) angiography showing vascular involvement in the aortic arch branches. There were total middle occlusion of the right subclavian artery, focal osteal stenosis with post-stenotic dilatation and diffuse long segmental severe stenosis of the right common carotid artery. There were irregular margins of the proximal portion and near total occlusion of the mid portion of the left common carotid artery. The patient also had severe proximal stenosis and total occlusion of the left subclavian artery after the origin of the left vertebral artery and severe proximal stenosis of the left vertebral artery

Measurements of BP in the four extremities by Doppler plethysmography were as follows: right arm SBP 73 mmHg, left arm SBP 58 mmHg, right ankle SBP 82 mmHg and left ankle SBP 139 mmHg. In our patient, both subclavian arteries are occluded and the right superficial femoral artery is occluded as well. No significant stenosis was present in the descending thoracic and abdominal aorta. Only left ankle BP reflects true SBP, which means she is normotensive.

Question 4

In patients with TA involving arch vessels, intervention is indicated in the case of:

A.Severe stenosis of the left subclavian artery without subclavian steal syndrome

B.Severe symptomatic stenosis

C.Frequent episodes of visual dimming

D.Recurrent episodes of transient ischaemic attack (TIA)

E.Severe dizziness

Becauseofherneurologicsymptomssuggestingamaurosisfugax,andseverenarrowingof all three cervical arteries, intervention to restore cerebral circulation was planned to lessen her cerebral ischaemic symptoms. Disease activity affects the long-term patency of any bypass or angioplasty procedure. Evaluation of disease activity of TA was performed.

Question 5

In patients with TA, disease activity can be assessed by:

A.  Presence of constitutional symptoms such as fever, malaise, arthralgia B.  Elevation of ESR or CRP level

C.  Carotid tenderness (carotodynia)

D.  Wall thickening or mural enhancement seen by CT or MR angiography E.  Increased uptake on positron emission tomography (PET)

In addition to the patient’s systemic symptoms of fever and malaise, both the ESR and CRP level were high and carotodynia was present. Carotid CT angiography showed concentric diffuse wall thickening, hyperenhancement of mural wall and a hypoattenuating inner ring of the artery (Fig. 35.3). An (18F) fluorodeoxyglucose (F-18 FDG) PET-CT scan

c

Fig. 35.3  Computed tomographic (CT) angiography. (a) Focal osteal stenosis is shown with poststenotic dilatation and diffuse long segmental severe stenosis of the right common carotid artery. There were irregular margins of the proximal portion and skipped lesions of near total occlusion of the proximal and mid-portion of the left common carotid artery. (b) Concentric wall thickening of both common carotid arteries with mural enhancement and low attenuation of the inner concentric ring.Thisprobablyrepresentslowattenuationoftheintima betweentheenhancedouter wallof the aorta and intraluminal opacified blood. (c) Thickened wall of the aortic arch