Clinical Epidemiology Unit, Department of Medicine, Karolinska University Hospital and Karolinska Institutet, Sweden; Department of Pediatrics, Örebro University Hospital, Sweden.
Department of Gastroenterology and Hepatology, Karolinska University Hospital and Karolinska Institutet, Sweden.
J Hepatol. 2014 Apr;60(4):802-8. doi: 10.1016/j.jhep.2013.11.017. Epub 2013 Nov 26.
BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is associated with increased mortality. Cardiovascular disease is a leading cause of death in the Western world. We examined the risk of cardiovascular disease and diabetes (type 1 and type 2) in patients with PSC and their first-degree relatives.
This prospective multicentre cohort study included 678 individuals with PSC diagnosed between 1970 and 2004, and 6347 non-PSC reference individuals matched for age, and sex. Through linkage of the Swedish Multigeneration Register we identified 3139 first-degree relatives to PSC patients and 30,953 first-degree relatives to the matched comparison cohort. We retrieved data on cardiovascular disease and type 1 and type 2 diabetes (T1D and T2D) from the National Patient Register, and then examined the association with PSC or having a family history of PSC using Poisson regression.
During 125,127 person-years of follow-up, 203 individuals with PSC had a diagnosis of cardiovascular disease. This corresponded to a 3.34-fold increased relative risk (RR) of cardiovascular disease in individuals with PSC (95% CI=2.86-3.91). The highest risk estimates were seen for diseases of the arteries, veins, and lymphatic vessels while the RR was neutral for ischemic heart disease (0.90) or only slightly elevated for cerebrovascular disease (1.74). Meanwhile, PSC first-degree relatives were at no increased risk of cardiovascular disease (RR=0.87; 95% CI=0.80-0.95). Individuals with PSC (RR=7.95; 95% CI=4.82-13.12), and to some extent also their first-degree relatives (RR=1.73; 95% CI=1.19-2.52) were at increased risk of T1D. Also for T2D were the RR is higher in individuals with PSC (RR=2.54; 95% CI=1.56-4.13) than in PSC first-degree relatives (RR=0.81; 95% CI=0.65-1.02).
PSC was associated with T1D, T2D, and non-ischemic cardiovascular disease. In contrast, first-degree relatives to PSC patients were only at a moderately increased risk of T1D, and at no increased risk of either cardiovascular disease or T2D.
原发性硬化性胆管炎(PSC)与死亡率升高相关。心血管疾病是西方国家死亡的主要原因。我们研究了 PSC 患者及其一级亲属患心血管疾病和糖尿病(1 型和 2 型)的风险。
本前瞻性多中心队列研究纳入了 1970 年至 2004 年间诊断为 PSC 的 678 名患者和年龄、性别相匹配的 6347 名非 PSC 参考个体。通过与瑞典多代人登记处的链接,我们确定了 3139 名 PSC 患者的一级亲属和 30953 名匹配对照组的一级亲属。我们从国家患者登记处检索了心血管疾病和 1 型和 2 型糖尿病(T1D 和 T2D)的数据,然后使用泊松回归检查 PSC 或家族史与 PSC 的关联。
在 125127 人年的随访期间,203 名 PSC 患者被诊断患有心血管疾病。这对应于 PSC 患者心血管疾病的相对风险增加了 3.34 倍(95%CI=2.86-3.91)。动脉、静脉和淋巴血管疾病的风险估计最高,而缺血性心脏病(RR=0.90)的风险估计中性,或脑血管疾病(RR=1.74)的风险估计仅略有升高。同时,PSC 一级亲属患心血管疾病的风险没有增加(RR=0.87;95%CI=0.80-0.95)。PSC 患者(RR=7.95;95%CI=4.82-13.12),在某种程度上,他们的一级亲属(RR=1.73;95%CI=1.19-2.52)患 1 型糖尿病的风险也增加。同样,PSC 患者(RR=2.54;95%CI=1.56-4.13)的 2 型糖尿病风险高于 PSC 一级亲属(RR=0.81;95%CI=0.65-1.02)。
PSC 与 1 型糖尿病、2 型糖尿病和非缺血性心血管疾病相关。相比之下,PSC 患者的一级亲属仅患 1 型糖尿病的风险适度增加,且患心血管疾病或 2 型糖尿病的风险没有增加。