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Clinical aspects

 

Autoimmune congenital heart block and primary Sjögren's syndrome: characterisation and outcomes of 49 cases


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18

 

  1. Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA-Sanitas, Barcelona, and Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Barcelona, Spain.
  2. Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil.
  3. Department of Internal Medicine, Hospital La Paz, Madrid, Spain.
  4. Department of Rheumatology, Skane University Hospital Malmö, Lund University, Lund, Sweden.
  5. Department of Oral and Maxillofacial Surgery, University of Groningen, University Medical Centre Groningen, the Netherlands.
  6. Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
  7. Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia.
  8. 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.
  9. Department of Rheumatology and Internal Medicine, Wroclaw Medical University, Wroclaw, Poland.
  10. Federal University of São Paulo, São Paulo, Brazil.
  11. Instituto Modelo de Cardiología Privado SRL, Córdoba, and Instituto Universitario de Ciencias Biomédicas de Córdoba, Córdoba, Argentina.
  12. Department of Statistics and Operations Research, Universitat Politècnica de Catalunya (UPC), Barcelona, Spain.
  13. Department of Rheumatology and Internal Medicine, Wroclaw Medical University, Wroclaw, Poland.
  14. 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.
  15. Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, the Netherlands.
  16. 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.
  17. 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
  18. 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.

Rheumatology Article