title: [Neonatal lupus syndrome: Literature review].
abstract: Neonatal lupus syndrome is associated with transplacental passage of maternal anti-SSA/Ro and anti-SSB/La antibodies. Children display cutaneous, hematological, liver or cardiac features. Cardiac manifestations include congenital heart block (CHB); endocardial fibroelastosis and dilated cardiomyopathy. The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is between 1 and 2% and the risk of recurrence is around 19%. Skin and systemic lesions are transient, whereas CHB is definitive and associated with significant morbidity and a mortality of 18%. A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or appear secondly. Mothers of children with CHB are usually asymptomatic or display Sjogren's syndrome or undifferentiated connective tissue disease. In anti-Ro/SSA positive pregnant women, fetal echocardiography should be performed at least every 2 weeks from the 16th to 24th week gestation. An electrocardiogram should be performed for all newborn babies. The benefit of fluorinated corticosteroid therapy for CHB detected in utero remains unclear. Maternal use of hydroxychloroquine may be associated with a decreased recurrent CHB risk in a subsequent offspring. A prospective study is actually ongoing to confirm these findings.
authors.affiliation: Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne pôle médecine, hôpital Cochin, AP-HP, université René-Descartes Paris V, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France.
authors.forename: N
authors.initials: N
authors.lastname: Morel
authors.affiliation: Service de médecine interne, hôpital Tenon, université Paris VI, 4, rue de la Chine, 75970 Paris cedex 20, France.
authors.forename: S
authors.initials: S
authors.lastname: Georgin-Lavialle
authors.affiliation: CHU Ste-Justine, université de Montréal, 3175, Côte Sainte-Catherine, Montréal H3T 1C5, Canada.
authors.forename: K
authors.initials: K
authors.lastname: Levesque
authors.affiliation: Service de médecine interne, CHU de Clermont-Ferrand, hôpital Gabriel-Montpied, université de Clermont-Ferrand, 58, rue Montalembert, 63003 Clermont-Ferrand cedex 1, France.
authors.forename: G
authors.initials: G
authors.lastname: Guettrot-Imbert
authors.affiliation: Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne pôle médecine, hôpital Cochin, AP-HP, université René-Descartes Paris V, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France.
authors.forename: V
authors.initials: V
authors.lastname: Le Guern
authors.affiliation: UE3C (unité d'explorations cardiologiques-cardiopathies congénitales), 31, avenue de Lowendal, 75015 Paris, France.
authors.forename: J
authors.initials: J
authors.lastname: Le Bidois
authors.affiliation: Unité de fœtopathologie, service histo-embryologie et cytogénétique, centre hospitalier universitaire Necker-Enfants-Malades, université Paris V, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: B
authors.initials: B
authors.lastname: Bessières
authors.affiliation: Laboratoire d'hématologie et CNRS UMR8147, hôpital Necker-Enfants-Malades, université René-Descartes, 149, rue de Sèvres, 75743 Paris cedex 15, France.
authors.forename: C
authors.initials: C
authors.lastname: Brouzes
authors.affiliation: Service de gynécologie obstétrique, Inserm UMR-781, centre hospitalier universitaire Necker-Enfants-Malades, université Paris V, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: D
authors.initials: D
authors.lastname: Le Mercier
authors.affiliation: Service de cardiologie pédiatrique, centre hospitalier universitaire Necker-Enfants-Malades, université Paris V, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: E
authors.initials: E
authors.lastname: Villain
authors.affiliation: Service de cardiologie pédiatrique, centre hospitalier universitaire Necker-Enfants-Malades, université Paris V, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: A
authors.initials: A
authors.lastname: Maltret
authors.affiliation: Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne pôle médecine, hôpital Cochin, AP-HP, université René-Descartes Paris V, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France. Electronic address: nathalie.costedoat@gmail.com.
authors.forename: N
authors.initials: N
authors.lastname: Costedoat-Chalumeau
mesh_terms: D000974:Antibodies, Antinuclear / Q000097:blood*; D005260:Female; D006327:Heart Block / Q000151:congenital* / Q000209:etiology / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000151:congenital* / Q000175:diagnosis / Q000276:immunology; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis / Q000276:immunology*
chemical_list: D000974:Antibodies, Antinuclear
keywords: Anti-La/SSB antibodies; Anti-Ro/SSA antibodies; Anticorps anti-SSA/Ro; Anticorps anti-SSB/La; Bloc auriculo-ventriculaire congénital; Congenital heart block; Gougerot-Sjögren; Lupus systémique; Lupus érythémateux néonatal; Neonatal lupus erythematosus; Sjögren's syndrome; Systemic lupus erythematosus