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Abstract Number: 2834

Maternal data Analysis Of The French Registry Of 205 Cases Of Immune Congenital Heart Block (neonatal lupus)

Kateri Levesque1, Nathalie Morel2, Gaëlle Guettrot-Imbert3, Mohamed Hamidou4, Jean Loup Pennaforte5, Pauline Orquevaux5, Jean-Charles Piette6, Christophe Deligny7, Zahir Amoura8, Olivier Meyer9, Olivier Fain10, Agathe Masseau11, Holly Bezanahary12, Pascal Cathebras13, Elizabeth Diot14, Yves Dulac15, Loic Guillevin16, Eric Hachulla17, Jean-Louis Pasquali18, Anne Besancon-Bergelin19, Bernard Bonnotte20, Jérome Lebidois21, Alice Maltret22, Elisabeth Villain22 and Nathalie Costedoat-Chalumeau23, 1Medicine Interne, Hopital Cochin, Paris, France, 2Internal Medicine, COCHIN, Paris, France, 3Internal Medicine, Centre Hospitalier de Clermont-Ferrand, Clermont-Ferrand, France, 4CHU Hôtel Dieu, Nantes, Nantes, France, 5Hu Robert Debre, CHU Reims, Reims, France, 6Department of Internal Medicine 1., CHU Pitié-Salpêtrière, Paris, France, 7Rhumatologie Et Médecine Interne, Centre hospitalier Universitaire de Fort de France, Fort de France, Martinique, 8Department of Internal Medicine 2. Referal center for SLE/APS, CHU Pitié-Salpêtrière, Paris, France, 9Rheumatology, Bichat University Hospital, Paris, France, 10Internal Medicine, Jean Verdier Hospital, Bondy, France, 11Internal Medicine Department, Nantes University Hospital, Nantes, France, 12Internal Medicine, University Hospital of Limoges, Limoges, France, 13Internal Medicine, University Hospital St Etienne, St Etienne, France, 14Department of Internal Medicine, Hôpital Bretonneau, Centre Hospitalier Régional Universitaire de Tours, Tours, France, Tours, France, 15University Hospital Toulouse, Paris, France, 16Internal Medicine, Hôpital Cochin, University Paris Descartes, Paris, France, 17Internal Medicine, University Hospital Lilles, Lille CEDEX, France, 18Strasbourg University, Hospital, CNRS UPR 3572, Strasbourg, France, 19Internal Medicine, Le Mans Hospital, Le Mans, France, 20Department of Internal Medicine, Centre Hospitalier de Dijon, Dijon, France, 21Cardiac Institute, Paris, France, 22Cardiology, Groupe Hospitalier Necker - Enfants Malades, Paris, France, 23Internal Medicine, Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: Heart disease, neonatal disorders and systemic lupus erythematosus (SLE)

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Session Information

Session Title: Systemic Lupus Erythematosus - Clinical Aspects: Pregnancy

Session Type: Abstract Submissions (ACR)

Background/Purpose: Congenital heart block (CHB) occurs in 1 to 2 % of pregnancies exposed to anti-SSA antibodies.  Few data are available regarding the risk of the mothers of fetuses/children with CHB to develop an autoimmune disease.

Methods: The inclusion criteria in the French registry of neonatal lupus are the positivity of anti-SSA and/or anti-SSB antibodies and a manifestation of neonatal lupus. In this retrospective study, we analyzed the data of the mothers who had a foetus/child with CHB.

Results: 184 mothers of 205 fetuses/children with CHB were included: 99.5% (183) had anti-SSA antibodies and 69.6% (128) had anti-SSB antibodies.
At the time of their first diagnosis of CHB, 136 mothers (73.9%) were asymptomatic. The 48 mothers (26.1%) with an auto-immune disease had a systemic lupus erythematosus (SLE, n=20, associated with antiphospholipid syndrome with venous thrombosis in 2 cases), a sjogren syndrome (n=12), an undifferentiated connective tissue disease (UCTD, n=10), a rheumatoid arthritis (n=3), a idiopathic thrombocytopenic purpura (n=1), an autoimmune hepatitis (n=1) and a systemic scleroderma (n=1). Clinical manifestations of SLE mainly included cutaneous and articular manifestations and only 8 mothers had more severe manifestations (renal, neurological, pericarditis, hematological). Only one had received cyclophosphamide.

After a median follow up period of 8.8 years [1 day-36.5 years], 75 mothers (40.8%) remained asymptomatic. The 109 mothers (59.2%) with an autoimmune disease had a Sjogren syndrome (n=42), an SLE (n=37, including 2 associated with an antiphospholipid syndrome), an UCTD (n=20), a rheumatoid arthritis (n=3), an SLE associated with a Sjogren syndrome (n=2), a idiopathic thrombocytopenic purpura (n=2), an autoimmune hepatitis (n=1), a systemic scleroderma (n=1) and a primary obstetrical antiphospholipid syndrome (n=1).

Conclusion: At the time of the CHB diagnosis in their offspring, a third of the mothers had a diagnosis of autoimmune disease. During the follow up period, another third developed an autoimmune disease. The remaining third was still asymptomatic after a median follow up period of 8.8 years [1 day-36.5 years].


Disclosure:

K. Levesque,
None;

N. Morel,
None;

G. Guettrot-Imbert,
None;

M. Hamidou,
None;

J. L. Pennaforte,
None;

P. Orquevaux,
None;

J. C. Piette,
None;

C. Deligny,
None;

Z. Amoura,
None;

O. Meyer,
None;

O. Fain,
None;

A. Masseau,
None;

H. Bezanahary,
None;

P. Cathebras,
None;

E. Diot,
None;

Y. Dulac,
None;

L. Guillevin,
None;

E. Hachulla,
None;

J. L. Pasquali,
None;

A. Besancon-Bergelin,
None;

B. Bonnotte,
None;

J. Lebidois,
None;

A. Maltret,
None;

E. Villain,
None;

N. Costedoat-Chalumeau,
None.

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