{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T23:28:47Z","timestamp":1788305327782,"version":"build-2803163510"},"reference-count":18,"publisher":"The Journal of Rheumatology","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2011,2]]},"abstract":"<jats:sec><jats:title>Objective.<\/jats:title><jats:p>Maternal anti-SSA\/Ro or anti-SSB\/La antibodies are associated with neonatal lupus erythematosus syndrome (NLES), especially congenital heart block (CHB), which may be associated with severe endocardial fibroelastosis (EFE) and dilated cardiomyopathy (DCM). A few reports have described severe EFE without CHB associated with anti-SSA\/Ro antibodies, with a poor prognosis. EFE has also been observed in biopsies of DCM that had been considered idiopathic. These points, considered in association with 5 unusual cases of mild EFE, led us to consider the relationship between underrecognized cases of isolated autoantibody-associated EFE and DCM that had been considered idiopathic.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods.<\/jats:title><jats:p>We analyzed 5 cases of EFE diagnosed <jats:italic>in utero<\/jats:italic> (n = 4) or after birth (n = 1). In 3 cases, maternal antibody status was discovered because of the EFE diagnosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>Endomyocardial hyperechogenicity predominated in the left atrium (n = 3) and mitral annulus (n = 3). No left-heart dysfunction was observed. Two mothers were treated with betamethasone. One mother chose to have a therapeutic abortion, and EFE was confirmed at autopsy. Electrocardiograms at birth (n = 4) did not show CHB. Other manifestations of NLES were present in all cases. One child had right ventricular hypoplasia and underwent a partial cavopulmonary anastomosis. At last followup (4\u20137 yrs), the other 3 children had normal heart function, and echocardiography showed a normal heart (n = 2) or mild persistent EFE (n = 1).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>Middle-term prognosis of isolated autoantibody-associated EFE may be better than previously reported, although the longterm prognosis remains unknown. We hypothesize that a fetal insult can lead to DCM.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.100317","type":"journal-article","created":{"date-parts":[[2010,11,16]],"date-time":"2010-11-16T05:14:47Z","timestamp":1289884487000},"page":"378-386","source":"Crossref","is-referenced-by-count":53,"title":["A New Presentation of Neonatal Lupus: 5 Cases of Isolated Mild Endocardial Fibroelastosis Associated with Maternal Anti-SSA\/Ro and Anti-SSB\/La Antibodies"],"prefix":"10.3899","volume":"38","author":[{"given":"GA\u00cbLLE","family":"GUETTROT-IMBERT","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"LAURENCE","family":"COHEN","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"LAURENT","family":"FERMONT","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"ELISABETH","family":"VILLAIN","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"CAMILLE","family":"FRANC\u00c8S","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"OLIVIER","family":"THIEBAUGEORGES","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"BERNARD","family":"FOLIGUET","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"GA\u00cbLLE","family":"LEROUX","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"PATRICE","family":"CACOUB","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"ZAHIR","family":"AMOURA","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"JEAN-CHARLES","family":"PIETTE","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"NATHALIE","family":"COSTEDOAT-CHALUMEAU","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2262","published-online":{"date-parts":[[2010,11,15]]},"reference":[{"key":"2018112909260040000_38.2.378.1","unstructured":"Buyon JP Clancy RM . 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