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Published on: February 8, 2019
Childhood-onset Takayasu arteritis: an update
Ashish J Mathew1, Ruchika Goel1, Sathish Kumar2
1Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India.
Insights
Childhood-onset Takayasu arteritis (c-TA) presents unique challenges, often diagnosed late with complications. Hypertension is the most common initial sign in pediatric patients.
Area of Science:
- Pediatric Rheumatology
- Vascular Inflammation
- Autoimmune Diseases
Background:
- Childhood-onset Takayasu arteritis (c-TA) is a rare, distinct vasculitis affecting children and adolescents.
- Limited global data exists for c-TA, differing significantly from adult Takayasu arteritis.
- Diagnosis is often delayed due to non-specific symptoms and lack of biomarkers, leading to irreversible complications.
Purpose of the Study:
- To describe the clinical characteristics and diagnostic approaches for childhood-onset Takayasu arteritis.
- To highlight the common presenting features and complications in a pediatric cohort.
- To emphasize the role of advanced imaging in diagnosing c-TA.
Main Methods:
- Retrospective analysis of 40 children diagnosed with c-TA.
- Correlation of clinical features with inflammatory markers for disease assessment.
- Utilization of advanced imaging, particularly magnetic resonance angiography, to minimize radiation exposure.
Main Results:
- Hypertension was the most frequent presenting feature in the cohort.
- Systemic symptoms including headache, fever, and weight loss were also observed.
- Diagnosis was often delayed, with many children presenting with established complications.
Conclusions:
- Childhood-onset Takayasu arteritis requires early recognition due to its distinct presentation and potential for severe complications.
- Hypertension is a key indicator for prompt evaluation in suspected pediatric cases.
- Advanced imaging modalities are crucial for accurate diagnosis while mitigating risks associated with radiation.
Abstract:
Childhood-onset Takayasu arteritis (c-TA) is a distinct subset affecting a wide age group, ranging from young infants to adolescents and it differs from adult TA in many aspects. There is scarcity of data on c-TA worldwide. The disease is classified using the European League Against Rheumatism/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society criteria. The non-specific nature of presenting complaints and lack of appropriate biomarkers delay the early diagnosis of this illness and many children present with complications, which become irreversible once they set in. One of the largest cohorts of 40 children with c-TA from our center reports hypertension as the commonest presenting feature. Systemic symptoms like headache, fever and weight loss are also described. Assessment of disease in c-TA is done by correlating clinical features with raised inflammatory markers. Advanced imaging plays an important role in diagnosis. In c-TA, the role of magnetic resonance angiography is advocated, taking into consideration the enormous amount of radiation exposure with other modalities. Complications of c-TA include cardiovascular, pulmonary, neurological and those arising secondary to long-term steroid and immunosuppression therapy.
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