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.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: AP-HP, Service de Médecine Interne, Centre de Référence National pour le Lupus Systémique et le Syndrome des Antiphospholipides, Centre Hospitalier Universitaire Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital, 75651 Paris Cedex 13, France.
authors.forename: Gaëlle
authors.initials: G
authors.lastname: Guettrot-Imbert
authors.forename: Laurence
authors.initials: L
authors.lastname: Cohen
authors.forename: Laurent
authors.initials: L
authors.lastname: Fermont
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Villain
authors.forename: Camille
authors.initials: C
authors.lastname: Francès
authors.forename: Olivier
authors.initials: O
authors.lastname: Thiebaugeorges
authors.forename: Bernard
authors.initials: B
authors.lastname: Foliguet
authors.forename: Gaëlle
authors.initials: G
authors.lastname: Leroux
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000276:immunology*; D004695:Endocardial Fibroelastosis / Q000151:congenital* / Q000276:immunology*; D005260:Female; D006801:Humans; D007118:Immunoassay; D008180:Lupus Erythematosus, Systemic / Q000151:congenital* / Q000276:immunology*; D011247:Pregnancy; D011296:Prenatal Diagnosis
chemical_list: D000974:Antibodies, Antinuclear