期刊
ANNALS OF THE RHEUMATIC DISEASES
卷 78, 期 5, 页码 696-703出版社
BMJ PUBLISHING GROUP
DOI: 10.1136/annrheumdis-2018-214406
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资金
- Swedish Research Council
- Swedish Rheumatism association
- King Gustaf the V:th 80-year foundation
- Heart-Lung Foundation
- Freemason's in Stockholm Foundation for Children's Welfare
- Stockholm County Council
- Karolinska Institute
- Torsten and Ragnar Soderberg Foundation
Objective 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. Methods Data 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. Results Individuals 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). Conclusions The 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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