Celiac disease (CD) and Budd-Chiari syndrome (BCS) are distinct medical conditions affecting different organ systems. However, reports suggest a potential association between them. This review examines existing literature and summarizes the current knowledge regarding the connection between CD and BCS. The pathophysiology of both conditions involves immune dysregulation and prothrombotic tendencies, though through different mechanisms. Several case reports and small studies indicate an increased incidence of BCS in individuals with CD, but the underlying mechanisms remain unclear. Proposed hypotheses include chronic inflammation, hypercoagulability, and potential genetic factors. However, more robust studies are needed to establish a definitive association and elucidate the shared pathophysiological factors between the two conditions. Management of CD primarily involves strict adherence to a gluten-free diet, leading to symptom improvement and normalization of serological markers. BCS requires a stepwise approach involving anticoagulation, endovascular therapy, and, in severe cases, liver transplantation. Recognizing the potential implications of the coexistence of CD and BCS is crucial for developing appropriate management strategies. Further research is warranted to investigate the potential association between CD and BCS, including large-scale epidemiological studies, genetic analyses, and mechanistic investigations. Understanding the underlying mechanisms and identifying risk factors for BCS development in individuals with CD will contribute to enhanced diagnostic capabilities, refined management strategies, and improved patient outcomes.
Copyright © 2025 The Author(s). Published by Wolters Kluwer Health, Inc.