Abstract
Vanishing bile duct syndrome is a rare clinical manifestation, and many clinicians tend to classify vanishing bile duct syndrome as a surgical disease and perform emergency surgery, leading to poor prognosis for patients. In this report, we present a case of a patient initially diagnosed with probable vanishing bile duct syndrome. However, through a meticulous step-by-step investigation, we ultimately determined that the patient was suffering from Brucella infection-induced hemophagocytic syndrome, which contributed to the development of the probable vanishing bile duct syndrome. Once a definitive diagnosis was established, the patient underwent treatment following anti-Brucella and Hemophagocytic lymphohistiocytosis-2004 protocols, leading to an improvement in the patient's condition. We conducted a literature review on brucellosis, and it demonstrated the lack of specificity in diagnosing Brucella infections and the diverse range of clinical manifestations. Failure to arrive at a definitive diagnosis may result in clinical misdiagnosis and delayed treatment, thereby leading to grave consequences.