Clinical study of children with Takayasu arteritis: a retrospective study from a single center in China

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.Electronic supplementary materialThe online version of this article (doi:10.1186/s12969-017-0164-2) contains supplementary material, which is available to authorized users.

Childhood‐onset Takayasu arteritis: A 15‐year experience from a tertiary referral center

“…Accordingly, in our cohort when segments and branches of the aorta were assessed individually, abdominal aorta was the most frequently affected part, followed by thoracic aorta, similar to a USA study . Abdominal aorta and renal artery are the most involved vessels in the majority of the aforementioned studies . Additionally, we found a higher frequency of left renal artery stenosis (50%) compared to the right (31.2%), which is in line with the rates reported by previous studies .…”

“…Female preponderance (75%) was in line with previous reports which documented the female frequency between 64% and 79% . Establishment of the diagnosis of Takayasu arteritis took a median of 2.5 months in our cohort, which was quite shorter compared to a previous Turkish cohort (median 12 months), and also compared to many previous reports.…”

“…In our study, the baseline high disease activity scores in PVAS, ITAS2010 and DEI.Tak decreased to a median of 0, 1 and 0 at last visits, respectively. Further, we have confirmed the good correlation and concordance between the three activity indices (ITAS2010, PVAS and DEI.Tak) in juvenile Takayasu arteritis, as previously described …”

“…In a South African study, the deaths were as a result of uncontrolled systemic hypertension and complications related to surgery . Retrospective cohort studies, which document no deceased patient, are currently not rare . In the last 10 years, only in a UK cohort was the mortality rate (27%) relatively high compared to other studies .…”

“…Similar to previous reports, constitutional symptoms were the most common complaints, accounting for 81.2% of our cohort. The most common physical examination finding in many studies is hypertension followed by blood pressure discrepancy or bruits . The most frequently detected abnormal findings at first admission in descending order were bruits (87.5%), blood pressure discrepancy (75%), decreased/absent pulse (75%) and systemic hypertension (62.5%).…”