- Case Report
- Open Access
Takayasu arteritis in children
© Al abrawi et al; licensee BioMed Central Ltd. 2008
- Received: 13 September 2007
- Accepted: 28 September 2008
- Published: 28 September 2008
Takayasu arteritis (TA) is a large vessel vasculitis that usually affects young female patients during the second and third decades of life, but has been reported in children as young as 24 months of age. Aim of this report was to describe four children (two girls) with TA, as well as summarizing main published studies. The mean age at presentation of our cases was 11 years (range 8–15). Three patients were Caucasians and one Asian. Arterial hypertension was the commonest mode of presentation followed by systemic symptoms. Other related symptoms were due to ischemia and consisted of abdomen, chest, and limb pain. An abdominal bruit was noted in only one patient. Inflammation markers were always abnormal. Angiography was performed in all cases; left subclavian artery and common carotid artery were more frequently involved. Renal artery stenosis was observed in two patients. One boy was diagnosed as having an associated immune deficiency (Wiskott-Aldrich syndrome). Treatment modalities included prednisone (n = 4), methotrexate (n = 3), and mycophenolate mofetil (MMF) (n = 1). Surgery was required in two patients. Follow-up ranged from 3 to 10 years since diagnosis. In three cases antihypertensive drugs and methotrexate were stopped, and prednisone was reduced to 7.5 mg/day.
- Juvenile Idiopathic Arthritis
- Mycophenolate Mofetil
- Renal Artery Stenosis
- Takayasu Arteritis
Takayasu arteritis (TA) is a large vessel vasculitis affecting mainly the aorta and its major branches. TA occurs most commonly in female patients in the second and third decades of life, but has also been reported in children as young as 24 months of age . The disease is more frequent in Asian populations, but has been reported in patients of all ethnical background. Descriptions of TA in the pediatric age are scanty. Moreover, treatment options have been limited so far, with few reports focusing on immunosuppressive treatment (2 methotrexate, 1 cyclophosphamide) in pediatric TA [2–4].
Characteristics of our patients with Takayasu arteritis.
Age at diagnosis (years)
Gender (M, male; F, female)
-pain left arm
-acute chest pain
-absent radial and brachial pulse, weak left carotid pulse
Laboratory findings at disease onset:
Treatment (initial dose):
-MMF (mg bid)
Bypass brachiocephalic trunk
Bypass abdominal aorta
Follow up (years)
Outcome and treatment at last visit
- Clinical remission
- Clinical remission
- Clinical remission
-prednisone 5 mg/d
-prednisone 7.5 mg/d
MMF 750 mg bid
-aspirin 100 mg/d
-prednisone 7.5 mg/d
Takayasu arteritis has been rarely reported in childhood. A common clinical mode of disease presentation in our patients was arterial hypertension (2/4 cases), together with nonspecific symptoms (headache, fatigue, myalgia, weight loss). Symptoms due to ischemia, which are frequent in adults, have been seldom reported in children. However, although true claudication (upon effort) was present only in one patient, we have observed ischemic findings (chest, limb, and abdominal pain) in three of our four patients. The disease is also called 'pulseless disease', since peripheral pulses are often absent due to vascular obstruction; however, this feature was present only in one of our patients. In addition, an abdominal bruit can often help in the diagnosis, but this was noted in only 1/4 of our cases.
Summary of main clinical features in our series and in published reports of pediatric Takayasu arteritis.
Elevated inflammatory parameters
Renal artery involvement
Abdominal aortic involvement
Thoracic aortic involvement
Ozen et al. 
Hong et al. 
Hahn et al. 
Jain et al. 
The diagnosis of TA is based on characteristic findings of diseased aorta and its major branches seen on angiography. This is demonstrated by luminal abnormalities such as stenosis or aneurysmal dilatation of the aorta, its major branches, and the pulmonary arteries. With regard to imaging studies, traditionally the angiographic patterns have been divided in: type I, affecting the aortic arch; type II, the thoracic and abdominal aorta; type III, the aorta both above and below the diaphragm; and type IV, the aorta and the pulmonary arteries. In our cases type I was the predominant pattern, whereas in two series type II was the predominant one [7, 9].
Vascular involvement during the disease course of our patients as investigated by imaging studies.
On treatment (2–5 years after diagnosis)
During a disease flare
At last visit
thrombosis left subclavian artery, left carotid thickening
stability of previous findings
After 6 years thickening of left carotid artery
stability of left carotid thickening
-reduction left subclavian artery diameter without thrombosis, hypoplastic left carotid without parietal lesions
angioMRI: thrombosis left common carotid artery, stenosis left subclavian artery, stenosis of the brachiocephalic artery, thrombosis descending thoracic aorta, right renal artery thrombosis and stenosis.
Echodoppler: (after surgery):
patency of left carotid and carotid trunk
normal visualization of the brachiocephalic trunk and right internal carotid artery
left carotid thrombosis, left subclavian stenosis, tronc brachiocephalic trunk stenosis
left subclavian stenosis, abdominal aortic stenosis, superior mesenteric artery stenosis, bilateral renal artery stenosis
stability subclavian lesion, improvement aortic wall involvement
stability of subclavian and aortic lesions
stenosis abdominal aorta
left subclavian thrombosis
With regard to treatment, in our series corticosteroids have been used in all cases, with the adjunction of methotrexate in three cases and MMF in one. Although the numbers are small and the retrospective nature of this report does not allow to draw firm conclusions, our patients have obtained clinical and laboratory remission with this regimen of early immunosuppression. Our patients were followed from 2 to 10 years since diagnosis (mean, 6 years) and medications at the last visit included only low-dose prednisone in three cases (one of whom still needed MMF and anti-hypertensives), while the fourth patient is now only on low-dose aspirin. Bypass surgery was required in two patients because of severe vascular occlusion, with excellent results as well. One bypass was carried out between the ascending aorta and the right carotid artery, the other one between the lower thoracic aorta and infra-renal aorta; in the latter a reimplantation of left renal artery and auto transplantation of right renal artery to common iliac was also performed.
Due to the rarity of the disease, there are no controlled studies of medical treatment of children with TA. An interesting new possibility is represented by the use of sildenafil, that has been recently reported in a 8-year old girl . We have been able to find in the literature only a previous French case, a 6 year-old girl who was treated with methotrexate . Methotrexate has been used both in adults and children with good results [16, 17]. Mycophenolate mofetil (MMF) has also recently been introduced in the treatment of adult patients with TA .
TA has been associated with other autoimmune diseases such as systemic lupus erythematosus, juvenile idiopathic arthritis, anterior uveitis, sarcoidosis, seronegative spondyloarthropathy, Crohn's disease, Wegener's granulomatosis, and Sweet syndrome [19–26]. One boy in our series, who suffered from eczema and thrombocytopenia since infancy, was diagnosed as having Wiskott-Aldrich syndrome (WAS) at the age of 16 years; this was subsequently confirmed by genetic study. The peculiar association of TA and WAS has already been mentioned in another previous report .
TA is a disease with severe prognosis, mortality rate being reported in children from 35 to 40% by five years . It is therefore important to have a high index of suspicion and in doubtful cases a low threshold for diagnostic evaluation. We underline the possibility of TA in any young patient with unexplained arterial hypertension.
We conclude that TA is not such a rare disease in a pediatric rheumatology setting, and also that it has to be considered in cases of unexplained arterial hypertension or unexplained inflammatory syndromes without signs of localization. A thorough physical examination can lead to the correct diagnosis if pulses cannot be felt or if an abdominal bruit is heard, even if these are not constant findings. Since the disease can be progressive and life-threatening, an early recognition is vital in order to start immunosuppression, which proved to be very successful in our patients.
We thank the patients or their parents, who gave permission to publish their clinical history.
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