Abstract
BackgroundDelayed diagnosis of childhood Takayasu arteritis (TA) is common due to its atypical symptoms. The objective of the present study was to summarize the clinical features of childhood TA to raise awareness and improve management.MethodsEleven children diagnosed with TA at our hospital were enrolled. Clinical information, diagnosis, treatment, and outcome were then examined retrospectively. The Pediatric Vasculitis Activity Score (PVAS) and the Indian Takayasu Clinical Activity Score (ITAS2010) were used to assess disease activity.ResultsMale-to-female ratio was 4:7. The mean age was 9.4 (1.4–14) years and the average time to diagnosis was 40.6 days (12–90 days). All patients suffered from hypertension and few had immunologic abnormalities. Two patients had low levels of autoantibodies and one had elevated immunoglobulin E levels. Aberrant (elevated) laboratory parameters included erythrocyte sedimentation rate (ESR) (9/10 patients, 90.0%), protein excretion (8/9 patients, 88.9%), renin-angiotensin-aldosterone system (RAAS) activity (5/5 patients, 100.0%), and serum lipid levels (3/5 patients, 60%). The common onset patterns were headache with convulsions (27.2%) and kidney damage (27.2%). The abdominal aorta (81.8%) and renal artery (72.7%) were the most commonly involved vessels. At presentation, the mean PVAS and ITAS2010 scores were 12.1 (6–26)/63 and 9.7 (5–14)/57, respectively. All patients were treated with glucocorticoids and antihypertensive agents; two underwent renal artery stent placement.ConclusionThe diagnosis of TA should be considered in patients with pediatric hypertension and high expression of inflammatory markers or abnormal urine results. Doppler ultrasonography of major vessels may be helpful. PVAS and ITAS2010 both help to evaluate disease activity, and the PVAS is recommended for patients with kidney damage. Glucocorticoid and antihypertensive agents are effective. Interventional therapy can be an option for patients with persistent hypertension.
Highlights
Delayed diagnosis of childhood Takayasu arteritis (TA) is common due to its atypical symptoms
Diagnostic criteria Patients were assessed according to the 2008 EULAR/ PRINTO/PRES criteria [5], which include angiographic abnormality of the aorta or its main branches and pulmonary arteries plus at least one of the following: (1) absence of the peripheral artery pulse or claudication induced by physical activity; (2) a >10 mm Hg difference in systolic BP in all four limbs; (3) Bruits over large arteries; (4) hypertension; and (5) increased levels of acute phase reactants (erythrocyte sedimentation rate(ESR) and/or C reactive protein(CRP)
General data The TA group was comprised of four boys (36.4%) and seven girls (63.6%)
Summary
Delayed diagnosis of childhood Takayasu arteritis (TA) is common due to its atypical symptoms. The objective of the present study was to summarize the clinical features of childhood TA to raise awareness and improve management. Many studies have attempted to identify new technologies that are both reliable and sensitive, for example, 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography-computed tomography (PET-CT) [4]. Such technologies have made diagnosis more timely and accurate, the most important factor that will yield an early diagnosis is an improvement in doctors’ clinical thinking and selection of the relevant tests/examinations. The aim is to help clinicians reach an early diagnosis and to improve the management of chronic vasculitis
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