Transplantation and Liver Surgery, Helsinki University Hospital and Helsinki University, Helsinki, Finland.
Division of Hepatology, Department of Upper GI, Karolinska University Hospital, Stockholm, Sweden; Department of Medicine, Huddinge, Karolinska Institutet, Stockholm, Sweden.
Clin Gastroenterol Hepatol. 2023 Apr;21(4):995-1004.e9. doi: 10.1016/j.cgh.2022.04.032. Epub 2022 May 13.
BACKGROUND & AIMS: Patients with Budd-Chiari syndrome (BCS) have an elevated risk of overall and liver-specific mortality, but this has not been quantified on a population level nor compared against a matched general population cohort.
We identified all patients in Sweden with a recorded diagnosis of BCS in the Swedish National Patient Register between 1987 and 2016. Patients with BCS were matched for age, sex, and municipality at baseline with up to 10 reference individuals from the general population. Data on cause-specific mortality were obtained from the Causes of Death Register. A Cox regression model was performed to investigate rates of all-cause and cause-specific mortality.
A total of 478 patients with BCS were matched with 4603 reference individuals. Of the patients with BCS, 43% were men, the median age was 58 years, 39% had a recorded diagnosis of a precipitating risk factor, and 13% had underlying liver disease. During a follow-up of up to 29 years, 243 (51%) of the patients with BCS died compared with 1346 (29%) of the reference individuals. Overall mortality was 70 per 1000 person-years in patients with BCS compared with 28 per 1000 person-years in reference individuals, translating into an adjusted hazard ratio (aHR) of 3.1 (95% confidence interval [CI], 2.6-3.6). Although liver-related mortality was particularly high (aHR, 47.6; 95% CI, 16.5-137.4), liver disease accounted for only 10% of deaths in BCS. The most common cause of death was cardiovascular disease (aHR, 2.2; 95% CI, 1.7-2.9).
Patients with BCS in Sweden had a 3-fold higher risk of death compared with general population reference individuals. Although mortality from liver diseases was high in relative terms, most patients died from cardiovascular causes.
布加氏综合征（BCS）患者的总体和肝脏特异性死亡率较高，但这尚未在人群水平上进行量化，也未与匹配的一般人群队列进行比较。
我们在瑞典国家患者登记处中确定了 1987 年至 2016 年间所有记录有 BCS 诊断的患者。在基线时，BCS 患者按年龄、性别和市与一般人群中的至多 10 名参考个体进行匹配。死因登记处提供了关于特定原因死亡率的数据。使用 Cox 回归模型研究全因和特定原因死亡率的发生率。
共纳入 478 例 BCS 患者，与 4603 名参考个体相匹配。BCS 患者中，43%为男性，中位年龄为 58 岁，39%有记录的诱发危险因素诊断，13%有潜在的肝脏疾病。在最长 29 年的随访期间，与 4603 名参考个体相比，243（51%）例 BCS 患者死亡。BCS 患者的总体死亡率为每 1000 人年 70 人，而参考个体为每 1000 人年 28 人，校正后的风险比（aHR）为 3.1（95%置信区间[CI]，2.6-3.6）。尽管肝脏相关死亡率特别高（aHR，47.6；95%CI，16.5-137.4），但肝脏疾病仅占 BCS 患者死亡人数的 10%。最常见的死亡原因是心血管疾病（aHR，2.2；95%CI，1.7-2.9）。
瑞典的 BCS 患者的死亡风险比一般人群参考个体高 3 倍。尽管从相对角度来看，肝脏疾病的死亡率较高，但大多数患者死于心血管疾病。
