Author Affiliations: Department of Cardiology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China (Zhu WH); Department of General Surgery, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310016, China (Shen LG); Institute of Radiology, Department of Paediatric Radiology, University Hospital Wuerzburg, 97080 Wuerzburg, Germany (Neubauer H)

Corresponding Author: Henning Neubauer, Helmuth-Zimmerer-Strasse 71, 97076 Wuerzburg, Germany (Tel: 0049-931-201-34715; Fax: 0049-931-201-34857; Email:  henning_neubauer@web.de)

Background: Pediatric patients with Takayasu arteritis were studied by analyzing clinical presentation, diagnostic images, response to multimodal therapy, and long-term outcome.

Methods: Fourteen consecutive children and adolescents (mean age: 10 years) were diagnosed with Takayasu arteritis at our institution between 1995 and 2007. They were subjected to clinical and diagnostic studies including color ultrasonography, MRI and angiography, and received interdisciplinary treatment.

Results: The median time lag between the first onset of symptoms and diagnosis was 7.7 weeks. The majority of patients presented with acute severe clinical symptoms and extensive vascular lesions. Hypertension was the most common finding on first presentation (93%), followed by headache (64%), nausea (64%) and palpitation (50%). Ten patients (71%) had reduced or absent carotid, brachial or femoral pulses in one or more locations. C-reactive protein was elevated in 79% of the patients and erythrocyte sedimentation rate in 64%. Cardiovascular imaging showed extensive vasculitis of both sides of the diaphragm in 86%. Complications included renal artery stenosis (n=7), aortic dissection, thoracic aortic aneurysm and infrarenal aneurysm (all n=1). Conservative drug treatment was effective in 50%. Interventional dilatation of stenosis and surgical therapy, including aortic bypass, resection of aneurysms and nephrectomy, were necessary in the remaining patients. Follow-up for 25 months to 12 years showed that all children are well without disease-related mortality.

Conclusions: Takayasu arteritis is a rare and potentially life-threatening disease in children, likely with a prolonged subclinical course. Rapid diagnosis and interdisciplinary management help to prevent life-threatening complications.

Key words: children; complications; diagnosis; Takayasu arteritis; therapy

World J Pediatr 2010;6(4):342-347