RT Journal Article
SR Electronic
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
FD The Journal of Rheumatology
SP 378
OP 386
DO 10.3899/jrheum.100317
VO 38
IS 2
A1 GAËLLE GUETTROT-IMBERT
A1 LAURENCE COHEN
A1 LAURENT FERMONT
A1 ELISABETH VILLAIN
A1 CAMILLE FRANCÈS
A1 OLIVIER THIEBAUGEORGES
A1 BERNARD FOLIGUET
A1 GAËLLE LEROUX
A1 PATRICE CACOUB
A1 ZAHIR AMOURA
A1 JEAN-CHARLES PIETTE
A1 NATHALIE COSTEDOAT-CHALUMEAU
YR 2011
UL http://www.jrheum.org/content/38/2/378.abstract
AB 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.