Childhood-onset systemic lupus erythematosus (cSLE) and malignancy: a nationwide multicentre series review

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorBRUFATTO, Matheus Zanata
dc.contributor.authorLANCAS, Sean Hideo Shirata
dc.contributor.authorFERNANDES, Taciana de Albuquerque Pedrosa
dc.contributor.authorSALLUM, Adriana Maluf Elias
dc.contributor.authorCAMPOS, Lucia Maria Arruda
dc.contributor.authorSAKAMOTO, Ana Paula
dc.contributor.authorTERRERI, Maria Teresa
dc.contributor.authorSZTAJNBOK, Flavio Roberto
dc.contributor.authorBICA, Blanca Elena Rios Gomes
dc.contributor.authorFERRIANI, Virginia Paes Leme
dc.contributor.authorCARVALHO, Luciana Martins de
dc.contributor.authorSILVA, Clovis Artur Almeida
dc.contributor.authorSAAD-MAGALHAES, Claudia
dc.date.accessioned2024-04-05T19:33:59Z
dc.date.available2024-04-05T19:33:59Z
dc.date.issued2024
dc.description.abstractBackgroundIncreased malignancy frequency is well documented in adult-systemic lupus erythematosus (SLE), but with limited reports in childhood-onset SLE (cSLE) series. We explored the frequency of malignancy associated with cSLE, describing clinical and demographic characteristics, disease activity and cumulative damage, by the time of malignancy diagnosis.MethodA retrospective case-notes review, in a nationwide cohort from 27 Pediatric Rheumatology centres, with descriptive biopsy-proven malignancy, disease activity/damage accrual, and immunosuppressive treatment were compiled in each participating centre, using a standard protocol.ResultsOf the 1757 cSLE cases in the updated cohort, 12 (0.7%) developed malignancy with median time 10 years after cSLE diagnosis. There were 91% females, median age at cSLE diagnosis 12 years, median age at malignancy diagnosis 23 years. Of all diagnosed malignancies, 11 were single-site, and a single case with concomitant multiple sites; four had haematological (0.22%) and 8 solid malignancy (0.45%). Median (min-max) SLEDAI-2 K scores were 9 (0-38), median (min-max) SLICC/ACR-DI (SDI) score were 1 (1-5) Histopathology defined 1 Hodgkin's lymphoma, 2 non-Hodgkin's lymphoma, 1 acute lymphoblastic leukaemia; 4 gastrointestinal carcinoma, 1 squamous cell carcinoma of the tongue and 1 anal carcinoma; 1 had sigmoid adenocarcinoma and 1 stomach carcinoid; 3 had genital malignancy, being 1 vulvae, 1 cervix and 1 vulvae and cervix carcinomas; 1 had central nervous system oligodendroglioma; and 1 testicle germ cell teratoma.ConclusionEstimated malignancy frequency of 0.7% was reported during cSLE follow up in a multicentric series. Median disease activity and cumulative damage scores, by the time of malignancy diagnoses, were high; considering that reported in adult series.eng
dc.description.indexMEDLINE
dc.description.indexPubMed
dc.description.indexScopus
dc.description.indexDimensions
dc.description.indexWoS
dc.description.sponsorshipCAPES
dc.identifier.citationADVANCES IN RHEUMATOLOGY, v.64, n.1, article ID 13, 7p, 2024
dc.identifier.doi10.1186/s42358-024-00353-3
dc.identifier.issn2523-3106
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/58949
dc.language.isoeng
dc.publisherBMCeng
dc.relation.ispartofAdvances in Rheumatology
dc.rightsopenAccesseng
dc.rights.holderCopyright BMCeng
dc.subjectChildhood-onset systemic lupus erithematosuseng
dc.subjectRegistryeng
dc.subjectMalignancyeng
dc.subjectLeukaemiaeng
dc.subjectLymphomaeng
dc.subjectSolid malignancyeng
dc.subject.otherdamage indexeng
dc.subject.othercancereng
dc.subject.otherriskeng
dc.subject.othercollegeeng
dc.subject.wosRheumatologyeng
dc.titleChildhood-onset systemic lupus erythematosus (cSLE) and malignancy: a nationwide multicentre series revieweng
dc.typearticleeng
dc.type.categoryrevieweng
dc.type.versionpublishedVersioneng
dspace.entity.typePublication
hcfmusp.author.externalBRUFATTO, Matheus Zanata:Sao Paulo State Univ UNESP, Botucatu Sch Med, Dept Internal Med, Discipline Nephrol, Botucatu, SP, Brazil
hcfmusp.author.externalLANCAS, Sean Hideo Shirata:Sao Paulo State Univ UNESP, Botucatu Sch Med, Dept Internal Med, Discipline Nephrol, Botucatu, SP, Brazil
hcfmusp.author.externalFERNANDES, Taciana de Albuquerque Pedrosa:Sao Paulo State Univ UNESP, Dept Pediat, Botucatu Med Sch, Botucatu, SP, Brazil
hcfmusp.author.externalSAKAMOTO, Ana Paula:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, Brazil
hcfmusp.author.externalTERRERI, Maria Teresa:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, Brazil
hcfmusp.author.externalSZTAJNBOK, Flavio Roberto:Univ Fed Rio De Janeiro, Pediat Rheumatol Unit, Rio De Janeiro, Brazil
hcfmusp.author.externalBICA, Blanca Elena Rios Gomes:Hosp Univ Clementino Fraga Filho, Univ Fed Rio De Janeiro, Pediat Rheumatol Unit, Rio De Janeiro, Brazil
hcfmusp.author.externalFERRIANI, Virginia Paes Leme:Univ Sao Paulo, Ribeirao Preto Med Sch, Dept Pediat, Ribeirao Preto, SP, Brazil
hcfmusp.author.externalCARVALHO, Luciana Martins de:Univ Sao Paulo, Ribeirao Preto Med Sch, Dept Pediat, Ribeirao Preto, SP, Brazil
hcfmusp.author.externalSAAD-MAGALHAES, Claudia:Sao Paulo State Univ UNESP, Botucatu Med Sch, Dept Pathol, Pediat Rheumatol Unit, Botucatu, SP, Brazil
hcfmusp.citation.scopus0
hcfmusp.contributor.author-fmusphcADRIANA MALUF ELIAS SALLUM
hcfmusp.contributor.author-fmusphcLUCIA MARIA MATTEI DE ARRUDA CAMPOS
hcfmusp.contributor.author-fmusphcCLOVIS ARTUR ALMEIDA DA SILVA
hcfmusp.description.articlenumber13
hcfmusp.description.issue1
hcfmusp.description.volume64
hcfmusp.origemWOS
hcfmusp.origem.dimensionspub.1168628176
hcfmusp.origem.pubmed38321580
hcfmusp.origem.scopus2-s2.0-85184434306
hcfmusp.origem.wosWOS:001157284400001
hcfmusp.publisher.cityLONDONeng
hcfmusp.publisher.countryENGLANDeng
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