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Takayasu arteritis in children
Safia Al Abrawi1, Marine Fouillet-Desjonqueres, Louis David
1Département de pédiatrie, Hôpital Edouard-Herriot and Université Claude-Bernard Lyon1, Lyon, France. r.cimaz@meyer.it.
Insights
Takayasu arteritis (TA), a large vessel vasculitis, can affect children, presenting with hypertension and ischemic pain. Early diagnosis and treatment with immunosuppressants and antihypertensives are crucial for managing this rare pediatric condition.
Area of Science:
- Pediatric Rheumatology
- Vascular Inflammation
- Autoimmune Diseases
Background:
- Takayasu arteritis (TA) is a rare large vessel vasculitis predominantly affecting young women.
- While typically diagnosed in the second and third decades of life, TA can manifest in childhood, even in patients as young as 24 months.
- Understanding pediatric TA is critical due to its potential for severe vascular complications.
Purpose of the Study:
- To describe four pediatric cases of Takayasu arteritis.
- To summarize key findings from published studies on childhood TA.
- To highlight clinical presentation, diagnostic features, and treatment outcomes in pediatric TA.
Main Methods:
- Retrospective case series of four children diagnosed with TA.
- Review of relevant published literature on pediatric Takayasu arteritis.
- Analysis of clinical data including age, ethnicity, symptoms, imaging, inflammatory markers, and treatment responses.
Main Results:
- The mean age of presentation in the four cases was 11 years (range 8-15).
- Common presentations included arterial hypertension and systemic symptoms, with ischemic pain in limbs, chest, and abdomen.
- Angiography revealed frequent involvement of the left subclavian and common carotid arteries; two patients had renal artery stenosis.
- One patient had an associated Wiskott-Aldrich syndrome, an immune deficiency.
Conclusions:
- Takayasu arteritis in children presents with diverse symptoms, often including hypertension and ischemic manifestations.
- Prompt diagnosis supported by imaging and inflammatory markers is essential for effective management.
- Treatment involves immunosuppressants and antihypertensives, with potential need for surgery, and long-term monitoring is required.
Abstract:
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.
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