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Rev Bras Reumatol Engl Ed. 2016 Mar-Apr;56(2):145-51. doi: 10.1016/j.rbre.2016.01.004. Epub 2016 Feb 18.

Brazilian multicenter study of 71 patients with juvenile-onset Takayasu's arteritis: clinical and angiographic features.

[Article in English, Portuguese]

Author information

1
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
2
Pediatric Rheumatology Sector, Instituto da Criança, Universidade de São Paulo, São Paulo, SP, Brazil.
3
Santa Casa de Misericórdia do São Paulo, São Paulo, SP, Brazil.
4
Medicine School, Universidade de São Paulo, Ribeirão Preto, SP, Brazil.
5
Universidade do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
6
Instituto de Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
7
Rheumatology Unit, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
8
Rheumatology Unit, Universidade Federal de Pernambuco, Recife, PE, Brazil.
9
Universidade Federal da Bahia, Salvador, BA, Brazil.
10
Hospital Pequeno Príncipe, Curitiba, PR, Brazil.
11
Sector of Pediatric Rheumatology, Department of Pediatrics, Universidade Federal de São Paulo, São Paulo, SP, Brazil. Electronic address: teterreri@terra.com.br.

Abstract

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.

CONCLUSION:

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.

KEYWORDS:

Adolescente; Arterite de Takayasu; Child; Criança; Image; Imagem; Takayasu's arteritis; Teenager

PMID:
27267528
DOI:
10.1016/j.rbre.2016.01.004
[Indexed for MEDLINE]
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