Clinical presentation and salivary gland histopathology of paediatric primary Sjogren's syndrome

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dc.contributor.authorSaad-Magalhaes, C. [UNESP]
dc.contributor.authorde Souza Medeiros, P. B.
dc.contributor.authorOliveira Sato, J.
dc.contributor.authorCustodio Domingues, M. A. [UNESP]
dc.contributor.institutionUniversidade Estadual Paulista (Unesp)
dc.date.accessioned2014-05-20T13:37:27Z
dc.date.available2014-05-20T13:37:27Z
dc.date.issued2011-05-01
dc.description.abstractObjectiveExplore the presentation, diagnostic criteria and exocrine gland histopathology of paediatric primary Sjogren's syndrome (PPSjS).MethodsA case series of 8 children is reported and American-European Consensus Group (AECG-2002) criteria were examined, as well as minor labial salivary and lachrymal gland biopsies, which were scored by a pathologist blinded to outcome. For all cases, connective tissue diseases and parotid-related infectious disease were excluded.ResultsAge at onset varied from 5-13 years old; 6 were females, all followed from diagnosis up to the last visit (1-10 years). The main features at presentation were recurrent tender parotid swelling and sialectasis imaging, with decreased salivary function assessed by Tc-99 scintigraphy. Mild sicca symptoms were observed in 4/8 cases. Systemic features, including fatigue, myalgia, arthritis, tenosynovitis, joint contractures, transient Raynaud's and high ESR, were recorded at onset. Autoantibody profile was unremarkable for diagnosis, while lymphocytic infiltration of labial salivary glands and sialectasis were observed in all biopsies (8/8). In lachrymal glands, massive lymphocytic infiltration and lymphocytic gastritis were observed during complementary assessment. Flares were treated with low dose steroids and long-term use of hydroxychloroquine (5/8), although only 318 fulfilled AECG-2002 diagnostic criteria, throughout the disease course.ConclusionPPSjS is rare, slowly progressive and its early presentation is variable. Standardised diagnostic algorithms should include recurrent parotid swelling and early diagnosis should rely mostly on salivary and lachrymal gland histopathology in this age group.en
dc.description.affiliationSão Paulo State Univ UNESP, Botucatu Med Sch, Paediat Rheumatol Unit, Dept Paediat, BR-18618970 São Paulo, Brazil
dc.description.affiliationSão Paulo State Univ UNESP, Botucatu Med Sch, Dept Pathol, BR-18618970 São Paulo, Brazil
dc.description.affiliationUnespSão Paulo State Univ UNESP, Botucatu Med Sch, Paediat Rheumatol Unit, Dept Paediat, BR-18618970 São Paulo, Brazil
dc.description.affiliationUnespSão Paulo State Univ UNESP, Botucatu Med Sch, Dept Pathol, BR-18618970 São Paulo, Brazil
dc.format.extent589-593
dc.identifierhttp://www.ncbi.nlm.nih.gov/pubmed/21640054
dc.identifier.citationClinical and Experimental Rheumatology. Pisa: Clinical & Exper Rheumatology, v. 29, n. 3, p. 589-593, 2011.
dc.identifier.issn0392-856X
dc.identifier.lattes7098310008371632
dc.identifier.orcid0000-0002-7631-7093
dc.identifier.urihttp://hdl.handle.net/11449/12961
dc.identifier.wosWOS:000293101500020
dc.language.isoeng
dc.publisherClinical & Exper Rheumatology
dc.relation.ispartofClinical and Experimental Rheumatology
dc.relation.ispartofjcr3.201
dc.rights.accessRightsAcesso restrito
dc.sourceWeb of Science
dc.subjectchildrenen
dc.subjectfocal sialadenitisen
dc.subjectrecurrent parotitisen
dc.subjectsialectasisen
dc.subjectSjogren's syndromeen
dc.titleClinical presentation and salivary gland histopathology of paediatric primary Sjogren's syndromeen
dc.typeArtigo
dcterms.rightsHolderClinical & Exper Rheumatology
unesp.author.lattes7098310008371632[1]
unesp.author.orcid0000-0002-7631-7093[1]
unesp.campusUniversidade Estadual Paulista (Unesp), Faculdade de Medicina, Botucatupt

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