Brazilian multicenter study of 71 patients with juvenile-onset Takayasu's arteritis: clinical and angiographic features.
OBJECTIVE
To describe the clinical and angiographic characteristics of Takayasu's arteritis in Brazilian children and adolescents.


METHODS
A retrospective data collection was performed in 71 children and adolescents followed in 10 Brazilian reference centers in Pediatric Rheumatology. The evaluation was carried out in three different time points: from onset of symptoms to diagnosis, from the 6th to 12th month of diagnosis, and in the last visit.


RESULTS
Of 71 selected patients, 51 (71.8%) were girls. The mean age of onset of symptoms and of time to diagnosis was 9.2 (±4.2) years and 1.2 (±1.4) years, respectively. At the end of the study, 20 patients were in a state of disease activity, 39 in remission and 5 had evolved to death. The most common symptoms in baseline assessment, second evaluation, and final evaluation were, respectively: constitutional, musculoskeletal, and neurological symptoms. A decrease in peripheral pulses was the most frequent cardiovascular signal, and an increase in erythrocyte sedimentation rate was the most frequent laboratory finding in all three evaluation periods. The tuberculin test was positive in 41% of those tested. Stenosis was the most frequent angiographic lesion, abdominal artery was the most affected segment, and angiographic type IV the most frequent. Most (90%) participants were treated with glucocorticoids, 85.9% required another immunosuppressive drug, and 29.6% underwent angioplasty.


CONCLUSIONS
This is the largest study on juvenile-onset Takayasu arteritis, and a high number of patients under the age of 10 years, with predominance of constitutional symptoms early in the disease, was observed.
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
Gleice
G
Clemente
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
Maria Odete
MO
Hilário
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
Claudio
C
Len
Pediatric Rheumatology Sector, Instituto da Criança, Universidade de São Paulo, São Paulo, SP, Brazil.
Clovis A
CA
Silva
Pediatric Rheumatology Sector, Instituto da Criança, Universidade de São Paulo, São Paulo, SP, Brazil.
Adriana M
AM
Sallum
Pediatric Rheumatology Sector, Instituto da Criança, Universidade de São Paulo, São Paulo, SP, Brazil.
Lúcia M
LM
Campos
Santa Casa de Misericórdia do São Paulo, São Paulo, SP, Brazil.
Silvana
S
Sacchetti
Santa Casa de Misericórdia do São Paulo, São Paulo, SP, Brazil.
Maria Carolina Dos
MC
Santos
Santa Casa de Misericórdia do São Paulo, São Paulo, SP, Brazil.
Andressa
A
Guariento Alves
Medicine School, Universidade de São Paulo, Ribeirão Preto, SP, Brazil.
Virgínia P
VP
Ferriani
Universidade do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
Flávio
F
Sztajnbok
Universidade do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
Rozana
R
Gasparello
Instituto de Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
Sheila
S
Knupp Oliveira
Instituto de Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
Marise
M
Lessa
Rheumatology Unit, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
Blanca
B
Bica
Rheumatology Unit, Universidade Federal de Pernambuco, Recife, PE, Brazil.
André
A
Cavalcanti
Universidade Federal da Bahia, Salvador, BA, Brazil.
Teresa
T
Robazzi
Hospital Pequeno Príncipe, Curitiba, PR, Brazil.
Marcia
M
Bandeira
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo, São Paulo, SP, Brazil. Electronic address: teterreri@terra.com.br.
Maria Teresa
MT
Terreri
D000293:Adolescent; D000792:Angiography / Q000379:methods*; D001938:Brazil; D002648:Child; D002675:Child, Preschool; D057510:Endovascular Procedures / Q000379:methods; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D062645:Percutaneous Coronary Intervention / Q000379:methods; D012189:Retrospective Studies; D013625:Takayasu Arteritis / Q000000981:diagnostic imaging* / Q000628:therapy
D005938:Glucocorticoids; D007166:Immunosuppressive Agents
Adolescente; Arterite de Takayasu; Child; Criança; Image; Imagem; Takayasu's arteritis; Teenager