Related Experiment Video
Updated: Sep 23, 2025
Competing-Risk Nomogram for Predicting Cancer-Specific Survival in Multiple Primary Colorectal Cancer Patients after Surgery
Published on: September 27, 2024
Three-fold Increased Risk of Death in Budd-Chiari Syndrome Compared to Matched Controls: A Population-based Cohort
Fredrik Åberg1, Rupesh Rajani2, Axel Wester3
1Transplantation and Liver Surgery, Helsinki University Hospital and Helsinki University, Helsinki, Finland.
Insights
Patients with Budd-Chiari syndrome (BCS) face a threefold increased mortality risk. Cardiovascular disease is the leading cause of death, not liver disease, in BCS patients.
Area of Science:
- Hepatology
- Epidemiology
- Public Health
Background:
- Budd-Chiari syndrome (BCS) is associated with increased mortality risk.
- Population-level mortality data for BCS patients compared to the general population is lacking.
Purpose of the Study:
- To quantify and compare overall and cause-specific mortality in BCS patients versus a matched general population cohort.
Main Methods:
- Swedish National Patient Register data (1987-2016) identified 478 BCS patients.
- BCS patients were matched with 4603 general population controls.
- Cox regression analysis assessed all-cause and cause-specific mortality rates.
Main Results:
- BCS patients had a 3.1-fold higher overall mortality risk (70 vs. 28 per 1000 person-years).
- Liver-related mortality was significantly elevated (aHR 47.6), but cardiovascular disease was the most common cause of death (aHR 2.2).
- 51% of BCS patients died during follow-up compared to 29% of controls.
Conclusions:
- BCS patients in Sweden exhibit a threefold increased risk of mortality compared to the general population.
- Cardiovascular disease, rather than liver disease, is the primary cause of death in BCS patients.
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.
Methods:
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.
Results:
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).
Conclusions:
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.
Related Concept Videos
01:17Chronic Bowel Disorders: Introduction
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
01:12Relative Risk
01:23Types of Biopharmaceutical Studies: Controlled and Non-Controlled Approaches
Non-controlled studies, commonly employed for initial exploration, lack a control group, rendering them susceptible to biases and external influences. In contrast,...
01:20Venous Thrombosis II: Clinical Manifestations and Diagnostic Studies
01:27Psychoneuroimmunology: Cardiovascular Disease
A key area of focus in PNI is the relationship between stress and coronary...
01:30Acute Coronary Syndrome III: Diagnostic Studies