Arteritis de Takayasu

Abstract

Takayasu's arteritis is a rare vasculitis in the pediatric population that affects the aorta and its branches. There are few studies with an appropriate number of patients and follow-up. To describe the clinical manifestations, laboratory alterations, radiological findings, and treatment in eight children and adolescents with Takayasu's arteritis. A retrospective analysis of patients' records from 1990 to 2001 was performed. There were six girls and two boys. The mean age at disease onset was five years and five months. The most common clinical manifestations were systemic findings and cardiovascular, dermatological and neurological abnormalities. In all patients inflammatory activity was high and in three patients the Mantoux test was strongly positive. The most common radiological findings were type IV and V. Treatment included steroids, methotrexate, cyclophosphamide, intravenous gamma globulin, and vascular surgery. Three patients presented sequelae. Takayasu's arteritis produces considerable morbidity and mortality. To make an early diagnosis, pediatricians should be aware of inflammatory systemic manifestations and cardiovascular abnormalities. To gain further knowledge of this entity prospective and ideally multicenter studies are required.