Childhood-onset Takayasu arteritis: an update

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.