Takayasu arteritis (TA) is a large vessel vasculitis rarely reported in children and infants. Most articles on pediatric TA have not focused on infants. We present the largest case series of infantile TA aiming to identify its demographic and clinical characteristics and compare them with existing data on older children.
We conducted an international multicentre retrospective cohort study. Epidemiological and clinical data were collected from patients' charts from six rheumatology centers. All patients met both the EULAR/PReS 2008 criteria and the 1990 ACR/EULAR criteria and were diagnosed with TA at age <5 years.
Twelve patients were included (50% female). Median age of symptom onset was 11 months, with a diagnostic delay of 4 months. The most common symptoms at presentation were hypertension, blood pressure differences between limbs, and fever. The most commonly involved arteries were the abdominal aorta and renal artery. Medications included steroids, conventional and biological disease-modifying antirheumatic drugs, and other immunosuppressive therapies. Half of the patients received biologic agents of which infliximab had the highest complete remission rate (40%). Other medications resulting in complete remission were cyclophosphamide (40%) and methotrexate (38%). Invasive procedures were required for 58% of patients. The most common complications were cardiac (50%), stroke (42%), and serious infections (33%). No patients died.
This study presents the largest series of infantile TA. Compared with other reported series on older children, infants with TA have more severe disease and were more likely to receive biologic agents, develop complications, and require invasive interventions.