S-128 1 Division of Paediatric Rheumatology, Dept. of Paediatrics, and 2 Dept. of Imaging Diagnostic Universidade Federal de São Paulo; 3 Paediatric Rheumatology Unit of Children’s Hospital, Universidade de São Paulo, Brazil; 4 Santa Casa de Misericórdia of São Paulo; 5 Faculdade de Medicina of Universidade de São Paulo - Ribeirão Preto, Brazil; 6 Universidade Estadual of Rio de Janeiro; 7 Institute of Paediatrics Martagão Gesteira of Universidade Federal do Rio de Janeiro; 8 Unit of Rheumatology of Universidade Federal do Rio de Janeiro, Brazil; 9 Unit of Rheumatology of Universidade Federal de Pernambuco, Recife, Brazil; 10 Universidade Federal da Bahia, Salvador; 11 Hospital Pequeno Príncipe, Curitiba, Brasil. Gleice Clemente, MD Maria Odete E. Hilário, MD Henrique Lederman, MD Clovis A. Silva, MD Adriana M. Sallum, MD Lúcia M. Campos, MD Silvana Sacchetti, MD Maria Carolina dos Santos, MD Virgínia P. Ferriani, MD Flávio Sztajnbok, MD Rozana Gasparello, MD Sheila Knupp Oliveira, MD Marise Lessa, MD Blanca Bica, MD André Cavalcanti, MD Teresa Robazzi, MD Marcia Bandeira, MD Maria Teresa Terreri, MD Please address correspondence to: Maria Teresa Terreri, MD, Division of Paediatric Rheumatology, Department of Paediatrics, Universidade Federal de São Paulo, Rua Ipê 112, ap111, 04022-005 São Paulo/SP, Brazil. E-mail: teterreri@terra.com.br Received on May 23, 2013; accepted in revised form on September 13, 2013. Clin Exp Rheumatol 2014; 32 (Suppl. 82): S128-S133. © Copyright CliniCal and ExpErimEntal rhEumatology 2014. Key words: Takayasu arteritis, vasculitis, child, adolescent, diagnosis, angiography Competing interests: none declared. ABSTRACT Objective. To evaluate and compare demographic, clinical, laboratory and angiographic data of Brazilian chil- dren and adolescents with Takayasu’s arteritis. Methods. In this Brazilian multicentre, retrospective study which included 10 paediatric rheumatology centres, we identiied 71 children and adolescents with Takayasu’s arteritis which were di- agnosed before their 19 th birthday. The patients’ demographic, clinical, labora- torial and angiographic data were re- corded. The participants were divided into two groups: children, deined by the WHO as younger than 10 years old (group 1: 36 patients) and adolescents, deined as individuals aged 10 to 19 years old (group 2: 35 patients). Fea- tures of both groups concerning disease manifestations were compared. Results. A total of 21 (58.3%) patients in group 1 and 30 (85.7%) patients in group 2 were girls (p=0.01). The mean age at disease onset, the mean time to diagnosis, and the mean follow-up time were 5.7 and 12.7, 1.8 and 0.7, 7.2 and 3.6 years, respectively, in groups 1 and 2 (p<0.001, 0.001 and <0.001). At ini- tial evaluation, constitutional symptoms (77.5%) were the most predominant symptoms and decreased peripheral pulses (85.9%) was the most predomi- nant clinical sign without differences between groups. The main laboratory indings were increased erythrocyte sedimentation rate followed by leuko- cytosis. Anaemia, thrombocytosis and higher platelet levels were signiicantly more frequent in group 1 (p=0.031, 0.001 and 0.018). Angiographic data were similar in both groups. Conclusion. Children presented more laboratory abnormalities but clinical and angiographic characteristics were similar to those presented by the ado- lescents. Diagnosis delay is longer in younger patients. Introduction Takayasu’s arteritis is a chronic vascu- litis that affects the wall of large and medium sized arteries, especially the aorta, its primary branches and the pul- monary artery. It was thought in the past to be a disease predominately af- fecting young Asian women but is cur- rently known to affect both genders, though most studies report a higher frequency among females (1-7). In the literature, an increasing number of papers on vasculitis, and particularly Takayasu’s arteritis, have been pub- lished in the last years (8-10). It is the third most frequent type of vasculitis in childhood. However, because it is a rare disease, there are few studies ad- dressing its prevalence among this age group. Some studies report a frequen- cy between 20% and 32% of patients younger than 20 years old (3, 11, 12). Lupi-Herrera et al. observed that 77% of the patients were aged between 10 and 20 years old (1). Studies report differences in the clini- cal presentation and prognosis of the disease between children and adults, as well as an important delay in the diag- nosis among paediatric patients (1, 4, 6, 11). There are some studies address- ing Takayasu’s arteritis in the paedi- atric population, but all studies are from speciic services, conducted with a small number of patients who were diagnosed according to adult criteria, which hinders acquiring knowledge concerning the disease during child- hood (13-16). The characteristics of the disease among different paediatric age Paediatric rheumatology Takayasu arteritis in a Brazilian multicentre study: children with a longer diagnosis delay than adolescents G. Clemente 1 , M.O.E. Hilário 1 , H. Lederman 2 , C.A. Silva 3 , A.M. Sallum 3 , L.M. Campos 3 , S. Sacchetti 4 , M.C. dos Santos 4 , V.P. Ferriani 5 , F. Sztajnbok 6 , R. Gasparello 6 , S. Knupp Oliveira 7 , M. Lessa 7 , B. Bica 8 , A. Cavalcanti 9 , T. Robazzi 10 , M. Bandeira 11 , M.T. Terreri 1