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

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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.


  • Pilar Brito-Zerón
  • Sandra G Pasoto
  • Angel Robles-Marhuenda
  • Thomas Mandl
  • Arjan Vissink
  • Berkan Armagan
  • Sonja Praprotnik
  • Gaetane Nocturne
  • Agata Sebastian
  • Virginia Fernandes Moça Trevisani
  • Soledad Retamozo
  • Nihan Acar-Denizli
  • Piotr Wiland
  • Antoni Sisó-Almirall
  • Hendrika Bootsma
  • Xavier Mariette
  • Manuel Ramos-Casals
  • Belchin Kostov
  • Sjögren Big Data Consortium
External organisations
  • La Paz Children's Hospital
  • Skåne University Hospital
  • Hanze University of Applied Sciences
  • Hacettepe University Department of Clinical Microbiology, Ankara
  • McMaster University Medical Centre
  • Center For Immunology Of Viral Infections And Autoimmune Diseases
  • Wrocław Medical University
  • Federal University of São Paulo
  • Hospital CIMA
  • University of São Paulo
  • Polytechnic University of Catalonia
  • University of Barcelona
  • University of Groningen
  • Institutd' Investigacions Biomèdiques August Pi iSunyer (IDIBAPS)
  • Instituto Universitario de Ciencias Biomédicas de Córdoba
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Rheumatology and Autoimmunity
Original languageEnglish
JournalClinical and Experimental Rheumatology
Publication statusE-pub ahead of print - 2020 Sep 30
Publication categoryResearch