Anatomical and pathological findings in hearts from fetuses and infants with cardiac manifestations of neonatal lupus

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dc.contributor.authorLlanos, Carolina
dc.contributor.authorFriedman, Deborah M.
dc.contributor.authorSaxena, Amit
dc.contributor.authorIzmirly, Peter M.
dc.contributor.authorTseng, Chung E.
dc.contributor.authorDische, Renata
dc.contributor.authorAbellar, Rosanna G.
dc.contributor.authorHalushka, Marc
dc.contributor.authorClancy, Robert M.
dc.contributor.authorBuyon, Jill P.
dc.date.accessioned2024-01-10T13:45:46Z
dc.date.available2024-01-10T13:45:46Z
dc.date.issued2012
dc.description.abstractObjective. The autopsy and clinical information on children dying with anti-SSA/Ro-associated cardiac manifestations of neonatal lupus (cardiac NL) were examined to identify patterns of disease, gain insight into pathogenesis and enhance the search for biomarkers and preventive therapies.
dc.description.abstractMethods. A retrospective analysis evaluating reports from 18 autopsies of cardiac NL cases and clinical data from the Research Registry for Neonatal Lupus was performed.
dc.description.abstractResults. Of the 18 cases with autopsies, 15 had advanced heart block, including 3 who died in the second trimester, 9 in the third trimester and 3 post-natally. Three others died of cardiomyopathy without advanced block, including two dying pre-natally and one after birth. Pathological findings included fibrosis/calcification of the atrioventricular (AV) node, sinoatrial (SA) node and bundle of His, endocardial fibroelastosis (EFE), papillary muscle fibrosis, valvular disease, calcification of the atrial septum and mononuclear pancarditis. There was no association of pathology with the timing of death except that in the third-trimester deaths more valvular disease and/or extensive conduction system abnormalities were observed. Clinical rhythm did not always correlate with pathology of the conduction system, and the pre-mortem echocardiograms did not consistently detect the extent of pathology.
dc.description.abstractConclusion. Fibrosis of the AV node/distal conduction system is the most characteristic histopathological finding. Fibrosis of the SA node and bundle of His, EFE and valve damage are also part of the anti-Ro spectrum of injury. Discordance between echocardiograms and pathology findings should prompt the search for more sensitive methods to accurately study the phenotype of antibody damage.
dc.description.funderNational Institutes of Health
dc.description.funderNIAMS
dc.description.funderS.L.E. Foundation NY Inc.
dc.description.funderAmerican Heart Association
dc.description.funderPfizer's Medical and Academic Partnerships programme
dc.description.funderNATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES
dc.fechaingreso.objetodigital2024-04-29
dc.format.extent7 páginas
dc.fuente.origenWOS
dc.identifier.doi10.1093/rheumatology/ker515
dc.identifier.eissn1462-0332
dc.identifier.issn1462-0324
dc.identifier.pubmedidMEDLINE:22308531
dc.identifier.urihttps://doi.org/10.1093/rheumatology/ker515
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/79078
dc.identifier.wosidWOS:000304200100022
dc.information.autorucMedicina;Llanos C;S/I;1007976
dc.issue.numero6
dc.language.isoen
dc.nota.accesocontenido parcial
dc.pagina.final1092
dc.pagina.inicio1086
dc.publisherOXFORD UNIV PRESS
dc.revistaRHEUMATOLOGY
dc.rightsacceso restringido
dc.subjectneonatal lupus
dc.subjectanti-SSA
dc.subjectRo antibodies
dc.subjectautoimmune congenital heart block
dc.subjectMATERNAL ANTI-RO
dc.subjectINTRAVENOUS IMMUNOGLOBULIN
dc.subjectRECURRENCE RATES
dc.subjectBLOCK
dc.subjectSSA/RO
dc.subjectRISK
dc.subjectMOTHERS
dc.subjectERYTHEMATOSUS
dc.subjectANTIBODIES
dc.subjectMULTICENTER
dc.subject.ods03 Good Health and Well-being
dc.subject.odspa03 Salud y bienestar
dc.titleAnatomical and pathological findings in hearts from fetuses and infants with cardiac manifestations of neonatal lupus
dc.typeartículo
dc.volumen51
sipa.codpersvinculados1007976
sipa.indexWOS
sipa.indexScopus
sipa.trazabilidadCarga SIPA;09-01-2024
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