Clinical aspects
Autoimmune congenital heart block and primary Sjögren's syndrome: characterisation and outcomes of 49 cases
P. Brito-Zerón1, S.G. Pasoto2, A. Robles-Marhuenda3, T. Mandl4, A. Vissink5, B. Armagan6, S. Praprotnik7, G. Nocturne8, A. Sebastian9, V. Fernandes Moça Trevisani10, S. Retamozo11, N. Acar-Denizli12, P. Wiland13, A. Sisó-Almirall14, H. Bootsma15, X. Mariette16, M. Ramos-Casals17, B. Kostov18
- Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA-Sanitas, Barcelona, and Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Barcelona, Spain.
- Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil.
- Department of Internal Medicine, Hospital La Paz, Madrid, Spain.
- Department of Rheumatology, Skane University Hospital Malmö, Lund University, Lund, Sweden.
- Department of Oral and Maxillofacial Surgery, University of Groningen, University Medical Centre Groningen, the Netherlands.
- Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
- Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia.
- Centre for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique - Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Université Paris Sud, INSERM, Paris, France.
- Department of Rheumatology and Internal Medicine, Wroclaw Medical University, Wroclaw, Poland.
- Federal University of São Paulo, São Paulo, Brazil.
- Instituto Modelo de Cardiología Privado SRL, Córdoba, and Instituto Universitario de Ciencias Biomédicas de Córdoba, Córdoba, Argentina.
- Department of Statistics and Operations Research, Universitat Politècnica de Catalunya (UPC), Barcelona, Spain.
- Department of Rheumatology and Internal Medicine, Wroclaw Medical University, Wroclaw, Poland.
- Primary Healthcare Transversal Research Group, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, and Primary Care Centre Les Corts, Consorci d’Atenció Primària de Salut Barcelona Esquerra (CAPSBE), Barcelona, Spain.
- Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, the Netherlands.
- Centre for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique - Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Université Paris Sud, INSERM, Paris, France.
- Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Barcelona; Department of Medicine, University of Barcelona; and Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain. mramos@clinic.cat
- Department of Statistics and Operations Research, Universitat Politècnica de Catalunya (UPC), Barcelona; Primary Healthcare Transversal Research Group, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona; and Primary Care Centre Les Corts, Consorci d’Atenció Primària de Salut Barcelona Esquerra (CAPSBE), Barcelona, Spain.
for the Sjögren Big Data Consortium
CER13890
2020 Vol.38, N°4 ,Suppl.126
PI 0095, PF 0102
Clinical aspects
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PMID: 33025893 [PubMed]
Received: 03/08/2020
Accepted : 10/09/2020
In Press: 30/09/2020
Published: 22/10/2020
Abstract
OBJECTIVES:
To characterise autoimmune congenital heart block (CHB) associated with a maternal diagnosis of primary Sjögren’s syndrome (pSS) confirmed either before, concomitant or after the first pregnancy complicated with CHB.
METHODS:
The following inclusion criteria were applied: (i) Mothers with positive Ro/La autoantibodies detected previously or at the time of diagnosis of the first case of CHB; (ii) diagnosis of CHB confirmed by fetal echocardiography; (iii) AV block diagnosed in uterus, at birth or within the neonatal period (0-27 days after birth) (8); (iv) absence of anatomical cardiac abnormalities which might be causal of AV block; and (v) maternal fulfillment of the 2002 SS criteria before, during or after having a pregnancy complicated with CHB.
RESULTS:
We identified 49 cases of autoimmune CHB in children born from 44 mothers who had a mean age at the time of pregnancy of 30.3 years (range 18 to 41). At the time of diagnosis of autoimmune CHB, all mothers had positive anti-Ro antibodies and 28/44 (64%) were positive for anti-La antibodies. Only 10 (22%) mothers with affected pregnancies had a diagnosis of primary SS at the time of diagnosis of the first pregnancy complicated by CHB (a mean of 4 years before, ranging from 1 to 10 years). In 6 (14%) mothers, primary SS was diagnosed during pregnancy or less than 12 months after the delivery/termination. In the remaining 28 (64%) mothers, pSS was confirmed 1-5 years after CHB diagnosis (n=19, 68%), 6-10 years after (n=2, 7%), or more than 10 years after the first case of CHB was diagnosed (n=7, 25%). CHB was diagnosed in uterus in all cases but two. AV block was initially incomplete in 11 fetuses and complete in 36 (no available data in 2 cases). Among the 35 (71%) surviving children with CHB, 5 (14%) developed other features of neonatal lupus. After the index pregnancy, 12 women had 20 subsequent pregnancies: five were complicated by a CHB (recurrence rate of CHB of 25%). The 4 women who had recurrent CHB were double-positive for anti-Ro and anti-La antibodies, and all had a confirmed pSS before having the first index case of CHB.
CONCLUSIONS:
In pSS, autoimmune CHB could be one of the first “indirect” signs of the disease in women of childbearing-age, in whom the diagnosis is confirmed several years later. Some maternal characteristics could be related with recurrent CHB, such as having an already-confirmed diagnosis of pSS and carrying the two Ro/La autoantibodies.