Download Takayasu Arteritis Associated with Hyperlipidemia presented with

Takayasu arteritis associated with
hypercholesterolemia presented with
abdominal mass in a 9 Year-old boy: case
report
Alyaa Kotby1, Ola A Elmasry1, Omneya I Youssef1, Mervat G
Mansour2, Abdul Rahman Shahein3
Address: 1 Division of Cardiology; 2 Pediatrics intensive care unit;
Department of Pediatrics, Children’s Hospital of Ain Shams
University, 38 Abbassia, Cairo, Egypt. 3 Department of Pediatrics,
555 University Avenue, Toronto, M5G 1X8, Canada.
Corresponding author email address:
[email protected]
ABSTRACT
Introduction: Takayasu arteritis (TA) is a large vessel granulomatous
vasculitis that commonl y affects the thoracic and abdominal aorta with
unknown etiology. The majorit y of TA occurs between 10 -40 years of age
and present with hypertension and absent right radial pulse. Case
presentation: We report a unique case of 9 year-old boy with angiogram proven Takayasu arteritis associated with hypercholesterolemia . The
initial clinical manifestations included high erythrocyte sedimentation
rate, right iliac fossa swelling and pulseless right lower limb. He
responded to oral steroids and surgical resection of the aneurysm.
Conclusion: The contribution of hypercholesterolemia in patients with TA
is not known and sho uld be investigated.
KEY WORDS: large vessel granulomatous disease, abdominal aorta,
angiogram, hypercholesterolemia, aneurysm, iliac fossa, pulseless disease
MANUSCRIPT TEXT
Introduction:
Takayasu arteritis was first diagnosed by a Japanese ophthalmologis t on
the year 1908. It is now considered a world wide disease. Takayasu
arteritis is a large vessel granulomatous vasculitis that commonl y affects
the thoracic and abdominal Aorta with unknown etiology. The mechanism
of arteritis has not been full y elucid ated to date but a non specific cell
mediated inflammatory process that progresses to fibrotic stenosis of the
aorta is widel y accepted
[1].
The disease passes into earl y inflammatory pre -pulseless and late arterial
occlusive pulseless phase. The inflamma tory stage usuall y presents with
nonspecific low grade fever, tachycardia and easy fatigabilit y with
hypertension
and
diminished
upper
extremities
pulses.
The
most
commonl y involved vessels include the left subclavian artery (50%), left
common carotid arte ry (20%), brachiocephalic trunk, renal arteries, celiac
trunk, superior mesenteric artery, and pulmonary arteries. Axillary,
vertebral, coronary and iliac arteries are infrequentl y involved
[2].
There have been many clinical reports of the disease and it s various
associations.
The
association
between
Takayasu's
arteritis
and
hyperlipidemia has not been described in the literature. In this case
report, the first case of childhood Takayasu's arteritis in combination with
h ypercholesterolemia is presented.
Case Presentation :
A 9 year-old white Caucasian Egyptian male child, the 4 t h born to
consanguineous parents presented with gradual onset of non-radiating
dull-aching pain in the right iliac fossa of one month duration. The pain
increased with active movem ent of the limb and decreased with resting.
There was neither history of trauma or back strain nor history of pervious
operations or hernia s. The patient had no fever, tachycardia, chest pain,
arthralgia, rashes or blurring of vision. Famil y history was fr ee of cardiac
or renal troubles and antenatal period passe d smoothl y with normal
records.
Physical examination revealed diminished right femoral, popliteal and
dorsalis pedis pulses with capillary perfusion from 2 -3 seconds. A deep
oval swelling in right iliac fossa measuring 5 centimeters in diameter was
palpated. The swelling was intra-abdominal, non-pulsating, firm in
consistency and tender with no fixation to the skin or muscle. Blood
pressure measured 111/72 (85 t h percentile) in the right upper limb.
A comprehensive radiological and laboratory assessment were done in
order to establish the diagnosis (Table 1). A color coded duplex
examination of the right lower limb arterial system showed a partiall y
thrombosed aneurysm of the right common iliac arter y with thrombus
matter occupying 50% of the arterial lumen and turbulent flow in the rest
of the lumen.
The rest of the arterial system was all patent , showing biphasic flow
pattern with average flow velocities and no evidence of arterial occlusion.
Left renal artery duplex showed mild stenosis with acceleration of 2.4
M/sec (N: > 3M/sec) and normal resistive index of 0.37 (N :< 0.7).
Electrocardiogram ( ECG) and chest X-ray were normal. Both right and
left lower limbs deep venous systems were thrombosed. Contrasted
computerized tomography and angiogram of the abdomen and pelvis
showed right common iliac artery aneurysm displacing the right psoas
muscle (Figure 1A and 1B).
A decision was made to surgicall y resect and bypass the thrombosed
aneurysm with saphenous graft. The limp perfusion and pain markedl y
improvement after the surgery. MRSA grown upon culturing the revealed
thrombus and was eradicated with intr avenous Vancom ycin 15mg/kg/dose
q8hrs over 14 days . The patient was then maintained on oral antico agulant
therapy (warfarin sodium 3mg/day) and cancelled his follow-up visits.
One year later, he presented with a picture of heart failure due to
hypertensive cardiomyopathy. Blood pressure measured 149/109 mmHg (>
95 t h percentile), 138/99 mmHg, 128/98 mmHg and 162/115 mmHg in right
upper limb, left upper limb, right lower limb and left lower limb
respectivel y. Echo cardiogram revealed left ventricular hypertrophy,
stenosed abdominal aorta with normal flow and anatom y of the coronaries.
The electrocardio gram (ECG) showed left heart axis deviation. Taking in
consideration the patient’s past history a thorough examination with a
(64) sixt y four multi -slice CT angiography for the aorta and both lower
limb arteries showed multiple stenotic segments of the aor ta and
brachiocephalic trunk with segments of relative dilatation. The left renal
artery couldn’t be delineated with complete occlusion of the right
common iliac artery (Figure 2). Lipid profile was done and repeatedl y
showed mild
hyperlipidemia
although
n ormal
dietetic
habits.
Both
erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were
again elevated.
Patient was then diagnosed to have Takayasu arteritis with an unusual
presentation based on the previousl y described angiograms , hypertensio n
and the markedl y elevated erythrocyte sedimentation rate .
Discussion:
Our patient first present ed with right iliac mass and pulseless right lower
limb which was preliminary thought to be a thrombosed congenital right
common iliac artery aneurysm. Elevated erythrocyte sedimentation rate
(ESR) and C-reactive protein (CRP) at this time were explained by the on
top infection. He improved after surgical resection and intravenous
antibiotics .
During the interim period between his first and second presentati on, the
disease seems to continue evolving with blood pressure climbing up until
precipitated heart fail ure (diastolic failure) . He was finally diagnosed
with takayasu arteritis ( TA) in his second presentation and fulfilled the
revised EULAR/PReS criteria
for TA, given (1) aortic and renal
narrowing on radiographic imaging, (2) undetectable right tibial pulses,
and (3) hypertension
[3].
The majorit y of TA cases occur between 10 -40 years of age
[4].
The
youngest age of presentation was reported by Weiss et al. about an
eighteen month -old infant with TA proven by arterial autopsy and
presented with cerebral aneurysm
[5].
TA is more common in females with
ratio varied from (6.6: 1) in Korea and (2.9: 1) in China as reported by
Park et al
[6]
and Zheng et al
[7]
respectively.
As a part of his work-up, fasting serum cholesterol level was done
repeatedl y and showed a moderate elevation 293 mg/dL (N: < 200).
Hypercholesterolemia couldn’t explain the above findings solel y. We
suggested this increase to contribute with the ongoing inflammation
affecting the major blood vessels with TA. But this explanation was not
studied before.
Parissis et al concluded in his study on a cohort of hypertensive patients
who proved to have elevated inflammatory markers in their blo od that
hypertension could induce an inflammatory process in blood vessels . He
also added that the coexistence of hypercholesterolemia may enhance this
inflammatory process .
[8].
TA is usuall y treated by Corticosteroids to achieve remission. Although
more than 50% of these patients flare with tapering
[9].
There are case
reports of treatment with methotrexate, azathioprine, infliximab
adalimumab
[12].
[10, 11]
and
The use of C yclophosphamide is reserved for severe
s ystemic involvement and frequent relapses
[13].
Once diagnosis of TA took place, the pati ent started on oral prednisolone
(2mg/kg/day divided doses ) in order to control disease activit y. Blood
pressure returned to the 75 t h centile on amlodepine, Carvidalol and
spironolactone.
His
lipid
profile
no rmalized
on
diet
control
and
atorvastatin (20 mg/day).
Assessing response to treatment in takayasu arteritis is challenging. Prior
studies have shown that clinical signs and symptoms of disease and
elevated acute phase reactants are poorl y correlated with disease activit y.
In the presence of hardware or clips from aneurismal repair, non -invasive
studies such as magnetic resonance angiogram ( MRA) and computerized
tomography angiogram ( CTA) are limited due to artifact. One recent
report discussed the utilit y of non-invasive measurement of aortic arterial
elastic properties with M -mode echocardiographic images in children with
TA
[14].
Furthermore, the optimal interval for serial imaging during
remission is uncertain.
Conclusion:
A question should be raised: Could hypercholesterolemia play a role in
Takayasu arteritis?
ACKNOWLEDGMENTS
I would like to express m y thankfulness and profound gratitude to m y
famil y and m y father professor “Hussein Shahein” for his support.
CONFLICTS OF INTEREST
“The author(s) declare that they have no competing interests’'.
SUPPORTING INFORMATION
There are two echocardiographs showing the stenosed abdominal aorta
below the diaphragm with a possibilit y of a plaque formation.
REFERENCES
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EULAR/PReS endorsed consensus criteria for the classification of
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in
an
18
month
old:
A
case
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FIGURES AND FIGURE CAPTIONS
Figure 1A Contrasted computerized tomography of the abdomen; a
roughl y rounded heterogeneous right iliac soft tissue densit y mass lesion
anterolateral t o L5 measuring 6x4x4 cm and displacing the right psoas
muscle
with
breaking
down
and
thrombosis
of
right
iliac
vein
(arrowhead). Figure 1B CT angiogram of the abdomen and pelvis; right
common iliac artery aneurysm (arrowhead).
Figure 2 Multi-slice CT angi ography for the aorta; marked irregular
thickening of the aortic wall affecting whole course of abdominal aorta,
till just proximal to the origin of inferior mesenteric artery with multiple
stenotic and relatively dilated segments (red arrowheads). Mild stenotic
segments are also noted at the brachiocephalic trunk, right subclavian and
both common carotid arteries (red arrowhead). Absent flow in righ t iliac
artery (blue arrowhead)
T AB LE S AN D T ABL E T IT LE
Table 1: Results of laboratory evaluation of the pat ient