Sjögren's syndrome in childhood.

To describe the clinical characteristics of juvenile Sjögren's syndrome (JSS) and report 5 new primary cases. Patients with SS whose disease began before age 16 were identified from a cohort study on SS. Previous patients with JSS published from 1952 to 1993 were found by literature review. Thi...

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Autores Principales: Anaya Cabrera, Juan Manuel, Ogawa, N., Talal N.
Formato: Artículo (Article)
Lenguaje:Inglés (English)
Publicado: The Journal of Rheumatology Publishing 1995
Materias:
Acceso en línea:https://repository.urosario.edu.co/handle/10336/28402
id ir-10336-28402
recordtype dspace
spelling ir-10336-284022020-08-28T15:48:07Z Sjögren's syndrome in childhood. Síndrome de Sjögren en la infancia. Anaya Cabrera, Juan Manuel Ogawa, N. Talal N. Sjögren's syndrome Childhood To describe the clinical characteristics of juvenile Sjögren's syndrome (JSS) and report 5 new primary cases. Patients with SS whose disease began before age 16 were identified from a cohort study on SS. Previous patients with JSS published from 1952 to 1993 were found by literature review. Thirty-nine adult patients with primary SS were selected as a control group. Five patients with primary JSS were identified and described. Thirty-four published primary JSS were reviewed. Altogether, there were 30 girls (77%). The mean age at onset was 7.8 +/- 4 years. Parotitis was the most common first symptom. An extraglandular manifestation (EGM) was the presenting feature in 9.3% of cases. Rheumatoid factor (RF) was positive in 71% and antinuclear antibodies (ANA) in 67%. During the course of disease, at least one EGM was noted in 20 cases (51%). Leukopenia was the most frequent of these (7/20, 35%). HLA-DR3 was observed in 4 of 6 cases in which HLA typing was done. Compared with adult primary SS, parotitis at onset was more frequent in children (62.5 vs 13%). The frequency of ANA and articular manifestations were higher in adults (67 vs 92%, and 15.4 vs 38.5%, respectively). We reviewed 31 cases of secondary JSS, of which the most common associated autoimmune disease was juvenile rheumatoid arthritis (42%). JSS preceded by years the associated autoimmune disease in nearly 50% of cases. JSS may be a common disease. Parotitis and the presence of RF and ANA are the main features at onset. Characteristics similar to those seen in adults are observed in children. Long-term followup is needed to assess outcome associated factors. 1995-06-01 2020-08-28T15:48:07Z info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion ISSN: 0315-162X EISSN: 1499-2752 https://repository.urosario.edu.co/handle/10336/28402 eng info:eu-repo/semantics/restrictedAccess application/pdf The Journal of Rheumatology Publishing Journal of Rheumatology
institution EdocUR - Universidad del Rosario
collection DSpace
language Inglés (English)
topic Sjögren's syndrome
Childhood
spellingShingle Sjögren's syndrome
Childhood
Anaya Cabrera, Juan Manuel
Ogawa, N.
Talal N.
Sjögren's syndrome in childhood.
description To describe the clinical characteristics of juvenile Sjögren's syndrome (JSS) and report 5 new primary cases. Patients with SS whose disease began before age 16 were identified from a cohort study on SS. Previous patients with JSS published from 1952 to 1993 were found by literature review. Thirty-nine adult patients with primary SS were selected as a control group. Five patients with primary JSS were identified and described. Thirty-four published primary JSS were reviewed. Altogether, there were 30 girls (77%). The mean age at onset was 7.8 +/- 4 years. Parotitis was the most common first symptom. An extraglandular manifestation (EGM) was the presenting feature in 9.3% of cases. Rheumatoid factor (RF) was positive in 71% and antinuclear antibodies (ANA) in 67%. During the course of disease, at least one EGM was noted in 20 cases (51%). Leukopenia was the most frequent of these (7/20, 35%). HLA-DR3 was observed in 4 of 6 cases in which HLA typing was done. Compared with adult primary SS, parotitis at onset was more frequent in children (62.5 vs 13%). The frequency of ANA and articular manifestations were higher in adults (67 vs 92%, and 15.4 vs 38.5%, respectively). We reviewed 31 cases of secondary JSS, of which the most common associated autoimmune disease was juvenile rheumatoid arthritis (42%). JSS preceded by years the associated autoimmune disease in nearly 50% of cases. JSS may be a common disease. Parotitis and the presence of RF and ANA are the main features at onset. Characteristics similar to those seen in adults are observed in children. Long-term followup is needed to assess outcome associated factors.
format Artículo (Article)
author Anaya Cabrera, Juan Manuel
Ogawa, N.
Talal N.
author_facet Anaya Cabrera, Juan Manuel
Ogawa, N.
Talal N.
author_sort Anaya Cabrera, Juan Manuel
title Sjögren's syndrome in childhood.
title_short Sjögren's syndrome in childhood.
title_full Sjögren's syndrome in childhood.
title_fullStr Sjögren's syndrome in childhood.
title_full_unstemmed Sjögren's syndrome in childhood.
title_sort sjögren's syndrome in childhood.
publisher The Journal of Rheumatology Publishing
publishDate 1995
url https://repository.urosario.edu.co/handle/10336/28402
_version_ 1676708439008477184
score 12,131701