Comorbidity and long-term outcome in patients with congenital heart block and their siblings exposed to Ro/SSA autoantibodies in utero

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Comorbidity and long-term outcome in patients with congenital heart block and their siblings exposed to Ro/SSA autoantibodies in utero

Mofors, J (author): Karolinska Institutet

Eliasson, H (author): Karolinska Institutet

Ambrosi, A (author): Karolinska Institutet

Salomonsson, S (author)

Skog, A (author): Karolinska Institutet

Fored, M (author): Karolinska Institutet

Ekbom, A (author): Karolinska Institutet

Bergman, G (author): Karolinska Institutet

Sonesson, SE (author): Karolinska Institutet

Wahren-Herlenius, M (author): Karolinska Institutet

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2019-02-26
2019
English.
In: Annals of the rheumatic diseases. - : BMJ. - 1468-2060 .- 0003-4967. ; 78:5, s. 696-703

Related links:: http://kipublication...; show more...; https://doi.org/10.1...; show less...

Journal article (peer-reviewed)

Abstract Subject headings

Congenital heart block (CHB) may develop in fetuses of Ro/SSA autoantibody-positive women. Given the rarity of CHB, information on comorbidity and complications later in life is difficult to systematically collect for large groups of patients. We therefore used nation-wide healthcare registers to investigate comorbidity and outcomes in patients with CHB and their siblings.MethodsData from patients with CHB (n= 119) and their siblings (n= 128), all born to anti-Ro/SSA-positive mothers, and from matched healthy controls (n= 1,190) and their siblings (n= 1,071), were retrieved from the Swedish National Patient Register. Analyses were performed by Cox proportional hazard modelling.ResultsIndividuals with CHB had a significantly increased risk of cardiovascular comorbidity, with cardiomyopathy and/or heart failure observed in 20 (16.8%) patients versus 3 (0.3%) controls, yielding a HR of 70.0 (95% CI 20.8 to 235.4), and with a HR for cerebral infarction of 39.9 (95% CI 4.5 to 357.3). Patients with CHB also had a higher risk of infections. Pacemaker treatment was associated with a decreased risk of cerebral infarction but increased risks of cardiomyopathy/heart failure and infection. The risk of systemic connective tissue disorder was also increased in patients with CHB (HR 11.8, 95% CI 4.0 to 11.8), and both patients with CHB and their siblings had an increased risk to develop any of 15 common autoimmune conditions (HR 5.7, 95% CI 2.83 to 11.69 and 3.6, 95% CI 1.7 to 8.0, respectively).ConclusionsThe data indicate an increased risk of several cardiovascular, infectious and autoimmune diseases in patients with CHB, with the latter risk shared by their siblings.

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By the author/editor: Mofors, J; Eliasson, H; Ambrosi, A; Salomonsson, S; Skog, A; Fored, M; show more...; Ekbom, A; Bergman, G; Sonesson, SE; Wahren-Herlenius ...; show less...

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