Sclerosing Cholangitis and Multiple Liver Abscesses in a Patient with Thymoma, Myasthenia Gravis, and Immune Deficiency (Good’s Syndrome)

BACKGROUND Thymoma is associated with immune deficiencies and can predispose to infections and autoimmune disorders, both of which can lead to hepatobiliary structural disease and infection. CASE REPORT We report the case of a 77-year-old woman with relapsed metastatic thymoma, myasthenia gravis, and hypogammaglobulinemia (Good's syndrome), who developed multiple liver abscesses and sclerosing cholangitis. She presented initially with gram-negative bacteremia with an undetermined source, and rapidly developed multiple liver abscesses. This injury pattern subsequently transformed into clinical and radiologic features of sclerosing cholangitis, with repeated episodes of bacteremia associated with biliary inflammation, identified by PET-CT, while persistent multiple hepatic filling defects remained quiescent. The unique clinical and radiologic features of this patient with both forme fruste liver abscesses and sclerosing cholangitis, are outlined, and the association of thymoma and immune deficiencies with this pattern of hepatobiliary disorder is discussed. Using the TriNetX federated global health research network that enables retrospective analysis of real-world clinical data from multiple health care organizations, we found that thymoma is associated with an increased risk of developing cholangitis. CONCLUSIONS This is the first report of both multiple liver abscesses and infected sclerosing cholangitis complicating thymoma in a patient with acquired immune deficiencies, suggesting that thymoma can predispose to infective hepatobiliary disorders, conceivably through its association with altered immunity and structural changes within the biliary system.