Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction that can lead to hepatic congestion, portal hypertension, ascites, esophageal varices, and liver failure. This syndrome is deeply associated with acquired or inherited hypercoagulation states; such as myeloproliferative disorders, factor V Leiden, deficiency of protein C, pregnancy, and systemic inflammation. Ulcerative colitis (UC) is an inflammatory bowel disease that leads to chronic systemic inflammation. Therefore, the aim of this study was to conduct a systematic literature review to analyze the association between Budd-Chiari syndrome and ulcerative colitis.

A systematic literature review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Papers were selected searching PubMed/Medline, SciELO, and LILACS databases in August 2021 using the search terms [Budd-Chiari syndrome] AND [ulcerative colitis]. The inclusion criterion was limited to observational studies, case reports, or case series that reported an association between Budd-Chiari syndrome and ulcerative colitis. Language was restricted to English, Spanish or Portuguese.

Among the 33 papers initially identified, 17 were eligible for this review after full texts were read. The selected studies were all case reports or case series. Budd-Chiari syndrome has been described as a rare extraintestinal complication of ulcerative colitis. Regarding gender, 12 patients were female and 5 were male. Seven articles reported cases of BCS in pediatric patients with UC. Three articles reported the diagnosis of BCS before the clinical manifestations and diagnosis of UC. Two cases also described the occurrence of severe thrombotic storm (development of multiple thrombi in different sites in quick succession) in patients with UC and no other coagulopathy.

The studies suggest that patients with ulcerative colitis have a higher risk of Budd-Chiari syndrome and thromboembolic events. This risk can be eight times higher during a flare-up, which can be explained by the increased systemic levels of cytokines (e.g. IL-1, 1L-6, and TNF-alpha) that can modulate the coagulation cascade leading to a hypercoagulation state. In addition, the increased intestinal epithelial permeability can promote bacterial translocation and systemic endotoxemia, which further accelerates the coagulation cascade activation. Still, studies have shown that patients with UC have higher rates of thrombocytosis, fibrinogen levels, and low antithrombin levels.

Budd-Chiari syndrome can be a rare extraintestinal complication of ulcerative colitis in pediatric and adult patients. This complication can occur even before the diagnosis of inflammatory bowel disease.