Infantile Takayasu: Demographic and Clinical Data from a Multi-Center Cohort
2Pediatric Rheumatology Unit, Rio de Janeiro State University
3Department of Pediatric Rheumatology, Hacettepe University
4Department of Pediatric Infectious Diseases and Immunology, Pontifical Catholic University of Chile
5Department of Pediatrics, Washington University in St. Louis/st. Louis Children's Hospital
6Pediatric Rheumatology Clinic, Kaplan Medical Center
Introduction: Takayasu arteritis (TA) is a large vessel vasculitis rarely reported in children, and its incidence is extremely low in infants. Most articles on pediatric takayasu have not focused on infants. We present the largest case series of infantile takayasu aiming to characterize demographic and clinical data and compare it with existing data on older children.
Methods: This was an international multi-center retrospective cohort study. Epidemiological and clinical data were collected from patient charts by doctors from six centers.
Results: Twelve patients meeting the ACR criteria of TA were included. Median age of symptom onset was 11 months, with a diagnostic delay of 4 months, 50% percent were female. The most common symptoms at presentation were hypertension, BP difference between upper and lower limbs, and fever. The arteries most commonly involved at diagnosis were the abdominal aorta, renal artery, and superior mesenteric artery. Different medications used included steroids, conventional and biological DMARDS, 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 and methothrexate. Invasive procedures were needed in 58% of patients. The most common complications were cardiac (50%), strokes (42%), and serious infections (33%). None of the patients died.
Conclusions: This study presents the largest series of infantile takayasu. Infants with TA were more likely to receive biologic agents, develop complications and need invasive interventions compared to older children in other reported series.