Department of Medicine, Karolinska Institutet, Stockholm, Sweden.
Department of Women's and Children's Health, Karolinska Universitetssjukhuset, Stockholm, Sweden.
Ann Rheum Dis. 2019 May;78(5):696-703. doi: 10.1136/annrheumdis-2018-214406. Epub 2019 Feb 26.
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.
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.
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).
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.
抗 Ro/SSA 自身抗体阳性女性的胎儿可能发生先天性心脏传导阻滞（CHB）。鉴于 CHB 的罕见性，很难系统地收集大量患者的合并症和后期并发症信息。因此，我们使用全国性的医疗保健登记处来调查 CHB 患者及其兄弟姐妹的合并症和结局。
从抗 Ro/SSA 阳性母亲所生的 119 例 CHB 患者及其 128 例兄弟姐妹（均患有 CHB）、1190 例匹配的健康对照者及其 1071 例兄弟姐妹（均未患有 CHB）中提取数据，这些数据均来自瑞典国家患者登记处。通过 Cox 比例风险模型进行分析。
患有 CHB 的个体发生心血管合并症的风险显著增加，20 例（16.8%）患者发生心肌病和/或心力衰竭，而 3 例（0.3%）对照组患者发生心肌病和/或心力衰竭，风险比为 70.0（95%CI 20.8 至 235.4），脑梗死的风险比为 39.9（95%CI 4.5 至 357.3）。CHB 患者还存在更高的感染风险。心脏起搏器治疗与脑梗死风险降低相关，但与心肌病/心力衰竭和感染风险增加相关。CHB 患者的系统性结缔组织疾病风险也增加（HR 11.8，95%CI 4.0 至 11.8），CHB 患者及其兄弟姐妹发生 15 种常见自身免疫性疾病的风险均增加（HR 分别为 5.7、95%CI 2.83 至 11.69 和 3.6、95%CI 1.7 至 8.0）。
数据表明，CHB 患者发生多种心血管、感染和自身免疫性疾病的风险增加，后者风险也存在于其兄弟姐妹中。
