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Clinical study of children with Takayasu arteritis: a retrospective study from a single center in China
Ye Feng1,2, Xuemei Tang3,4,5, Mingyue Liu1,2
1Department of Rheumatology and Immunology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, No. 136, Zhongshan 2nd Road, Yuzhong District, Chongqing, 400014, People's Republic of China.
Insights
Childhood Takayasu arteritis (TA) often presents with atypical symptoms, leading to delayed diagnosis. This study highlights key clinical features, diagnostic markers, and effective treatments for pediatric TA to improve patient outcomes.
Area of Science:
- Pediatric Rheumatology
- Vascular Inflammation
- Autoimmune Diseases
Background:
- Childhood Takayasu arteritis (TA) diagnosis is frequently delayed due to nonspecific symptoms.
- Early recognition and management are crucial for preventing long-term complications.
Purpose of the Study:
- To summarize the clinical manifestations of pediatric Takayasu arteritis.
- To enhance awareness and guide the management of childhood TA.
Main Methods:
- Retrospective analysis of 11 pediatric patients diagnosed with TA.
- Evaluation of clinical data, diagnostic parameters, treatment strategies, and outcomes.
- Assessment of disease activity using Pediatric Vasculitis Activity Score (PVAS) and Indian Takayasu Clinical Activity Score (ITAS2010).
Main Results:
- Hypertension was present in all patients; common onset patterns included headache with convulsions and kidney damage.
- The abdominal aorta and renal arteries were the most frequently affected vessels.
- Elevated ESR, protein excretion, RAAS activity, and lipid levels were observed; mean PVAS and ITAS2010 scores indicated significant disease activity.
Conclusions:
- Pediatric hypertension with inflammatory markers or abnormal urinalysis suggests TA.
- PVAS and ITAS2010 are valuable for assessing disease activity, with PVAS recommended for kidney involvement.
- Glucocorticoids and antihypertensives are effective; interventional therapy may be considered for persistent hypertension.
Background:
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.
Methods:
Eleven 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.
Results:
Male-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.
Conclusion:
The 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.
