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Autoimmune congenit...
Autoimmune congenital heart block and primary Sjögren's syndrome : characterisation and outcomes of 49 cases
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- Brito-Zerón, Pilar (author)
- Hospital CIMA
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- Pasoto, Sandra G (author)
- University of São Paulo
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- Robles-Marhuenda, Angel (author)
- La Paz Children's Hospital
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- Mandl, Thomas (author)
- Lund University,Lunds universitet,Internmedicin - epidemiologi,Forskargrupper vid Lunds universitet,Internal Medicine - Epidemiology,Lund University Research Groups,Skåne University Hospital
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- Vissink, Arjan (author)
- Hanze University of Applied Sciences
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- Armagan, Berkan (author)
- Hacettepe University
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- Praprotnik, Sonja (author)
- McMaster University Medical Centre
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- Nocturne, Gaetane (author)
- Center For Immunology Of Viral Infections And Autoimmune Diseases
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- Sebastian, Agata (author)
- Wrocław Medical University
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- Fernandes Moça Trevisani, Virginia (author)
- Federal University of São Paulo
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- Retamozo, Soledad (author)
- Instituto Universitario de Ciencias Biomédicas de Córdoba
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- Acar-Denizli, Nihan (author)
- Polytechnic University of Catalonia
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- Wiland, Piotr (author)
- Wrocław Medical University
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- Sisó-Almirall, Antoni (author)
- Institutd' Investigacions Biomèdiques August Pi iSunyer (IDIBAPS)
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- Bootsma, Hendrika (author)
- University of Groningen
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- Mariette, Xavier (author)
- Center For Immunology Of Viral Infections And Autoimmune Diseases
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- Ramos-Casals, Manuel (author)
- University of Barcelona
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- Kostov, Belchin (author)
- Polytechnic University of Catalonia
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(creator_code:org_t)
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- 2020
- 2020
- English.
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In: Clinical and Experimental Rheumatology. - 0392-856X. ; 38:Suppl 126, s. 95-102
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Abstract
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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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Reumatologi och inflammation (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Rheumatology and Autoimmunity (hsv//eng)
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Brito-Zerón, Pil ...
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Pasoto, Sandra G
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Robles-Marhuenda ...
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Mandl, Thomas
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Vissink, Arjan
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Armagan, Berkan
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Praprotnik, Sonj ...
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Nocturne, Gaetan ...
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Sebastian, Agata
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Fernandes Moça T ...
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Retamozo, Soleda ...
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Acar-Denizli, Ni ...
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Wiland, Piotr
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Sisó-Almirall, A ...
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Bootsma, Hendrik ...
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Mariette, Xavier
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Ramos-Casals, Ma ...
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Kostov, Belchin
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Lund University