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)

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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