TY - JOUR T1 - A New Presentation of Neonatal Lupus: 5 Cases of Isolated Mild Endocardial Fibroelastosis Associated with Maternal Anti-SSA/Ro and Anti-SSB/La Antibodies JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.100317 SP - jrheum.100317 AU - Gaëlle Guettrot-Imbert AU - Laurence Cohen AU - Laurent Fermont AU - Elisabeth Villain AU - Camille Francès AU - Olivier Thiebaugeorges AU - Bernard Foliguet AU - Gaëlle Leroux AU - Patrice Cacoub AU - Zahir Amoura AU - Jean-Charles Piette AU - Nathalie Costedoat-Chalumeau Y1 - 2010/11/15 UR - http://www.jrheum.org/content/early/2010/11/12/jrheum.100317.abstract N2 - Objective 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. Methods We analyzed 5 cases of EFE diagnosed in utero (n = 4) or after birth (n = 1). In 3 cases, maternal antibody status was discovered because of the EFE diagnosis. Results 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 (4–7 yrs), the other 3 children had normal heart function, and echocardiography showed a normal heart (n = 2) or mild persistent EFE (n = 1). Conclusion 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. ER -