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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Fecha de publicación:
1995
Institución:
Universidad del Rosario
Repositorio:
Repositorio EdocUR - U. Rosario
Idioma:
eng
OAI Identifier:
oai:repository.urosario.edu.co:10336/28402
Acceso en línea:
https://repository.urosario.edu.co/handle/10336/28402
Palabra clave:
Sjögren's syndrome
Childhood
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Restringido (Acceso a grupos específicos)
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oai_identifier_str oai:repository.urosario.edu.co:10336/28402
network_acronym_str EDOCUR2
network_name_str Repositorio EdocUR - U. Rosario
repository_id_str
spelling 194747786006e84280f-9562-41a7-bc79-65a3191228f2-122a3bd8c-89b4-4c98-b36a-7b77243faeff-12020-08-28T15:48:07Z2020-08-28T15:48:07Z1995-06-01To 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.application/pdfISSN: 0315-162XEISSN: 1499-2752https://repository.urosario.edu.co/handle/10336/28402engThe Journal of Rheumatology Publishing1158No. 61152Journal of RheumatologyVol. 22Journal of Rheumatology, ISSN: 0315-162X; EISSN: 1499-2752, Vol.22, No.6 (June, 1995); pp. 1152 - 1158https://pubmed.ncbi.nlm.nih.gov/7674246/Restringido (Acceso a grupos específicos)http://purl.org/coar/access_right/c_16ecJournal of Rheumatologyinstname:Universidad del Rosarioreponame:Repositorio Institucional EdocURSjögren's syndromeChildhoodSjögren's syndrome in childhood.Síndrome de Sjögren en la infancia.articleArtículohttp://purl.org/coar/version/c_970fb48d4fbd8a85http://purl.org/coar/resource_type/c_6501Anaya, Juan-ManuelOgawa, N.Talal N.10336/28402oai:repository.urosario.edu.co:10336/284022021-06-03 00:49:47.65https://repository.urosario.edu.coRepositorio institucional EdocURedocur@urosario.edu.co
dc.title.spa.fl_str_mv Sjögren's syndrome in childhood.
dc.title.TranslatedTitle.spa.fl_str_mv Síndrome de Sjögren en la infancia.
title Sjögren's syndrome in childhood.
spellingShingle Sjögren's syndrome in childhood.
Sjögren's syndrome
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.
dc.subject.keyword.spa.fl_str_mv Sjögren's syndrome
Childhood
topic Sjögren's syndrome
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.
publishDate 1995
dc.date.created.spa.fl_str_mv 1995-06-01
dc.date.accessioned.none.fl_str_mv 2020-08-28T15:48:07Z
dc.date.available.none.fl_str_mv 2020-08-28T15:48:07Z
dc.type.eng.fl_str_mv article
dc.type.coarversion.fl_str_mv http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.coar.fl_str_mv http://purl.org/coar/resource_type/c_6501
dc.type.spa.spa.fl_str_mv Artículo
dc.identifier.issn.none.fl_str_mv ISSN: 0315-162X
EISSN: 1499-2752
dc.identifier.uri.none.fl_str_mv https://repository.urosario.edu.co/handle/10336/28402
identifier_str_mv ISSN: 0315-162X
EISSN: 1499-2752
url https://repository.urosario.edu.co/handle/10336/28402
dc.language.iso.spa.fl_str_mv eng
language eng
dc.relation.citationEndPage.none.fl_str_mv 1158
dc.relation.citationIssue.none.fl_str_mv No. 6
dc.relation.citationStartPage.none.fl_str_mv 1152
dc.relation.citationTitle.none.fl_str_mv Journal of Rheumatology
dc.relation.citationVolume.none.fl_str_mv Vol. 22
dc.relation.ispartof.spa.fl_str_mv Journal of Rheumatology, ISSN: 0315-162X; EISSN: 1499-2752, Vol.22, No.6 (June, 1995); pp. 1152 - 1158
dc.relation.uri.spa.fl_str_mv https://pubmed.ncbi.nlm.nih.gov/7674246/
dc.rights.coar.fl_str_mv http://purl.org/coar/access_right/c_16ec
dc.rights.acceso.spa.fl_str_mv Restringido (Acceso a grupos específicos)
rights_invalid_str_mv Restringido (Acceso a grupos específicos)
http://purl.org/coar/access_right/c_16ec
dc.format.mimetype.none.fl_str_mv application/pdf
dc.publisher.spa.fl_str_mv The Journal of Rheumatology Publishing
dc.source.spa.fl_str_mv Journal of Rheumatology
institution Universidad del Rosario
dc.source.instname.none.fl_str_mv instname:Universidad del Rosario
dc.source.reponame.none.fl_str_mv reponame:Repositorio Institucional EdocUR
repository.name.fl_str_mv Repositorio institucional EdocUR
repository.mail.fl_str_mv edocur@urosario.edu.co
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