P3D253: Case of Vancomycin-responsive Peripheral Blood Eosinophilia in a Child with Ulcerative Colitis with Autoimmune Hepatitis and Primary Sclerosing Cholangitis

Introduction: Extraintestinal manifestations in inflammatory bowel disease (IBD) are common, especially in patients with ulcerative colitis (UC). Primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH), together described as overlap syndrome, are rare extraintestinal manifestations that have high mortality rates and are difficult to manage. In addition to immunosuppressants and immunomodulators used to manage IBD, other therapies are needed to control for symptoms of hepatic disease. In specific cases, oral vancomycin is used to prevent cholangitis (Davies, et al). The combination of these therapies predispose patients to numerous complications, including infections and cancer. Abnormalities such as peripheral blood eosinophilia (PBE) need to be thoroughly worked up to rule out infections, neoplasms, and drug-induced adverse reactions. The following is a case of PBE with cholangitis in a child with UC and overlap syndrome that resolved with reintroduction of oral vancomycin.

Case Description: The patient is a 10-year-old female who initially presented with hematochezia, hepatitis, and coagulopathy, and was diagnosed with UC, PSC and AIH. Her disease was controlled in remission on azathioprine and mesalamine. Oral vancomycin was added as prophylaxis and continued chronically due to recurrent bouts of cholangitis. After 5 years of prophylactic therapy, vancomycin was weaned off. Within 5 months of discontinuing vancomycin, she exhibited findings of early cholangitis and severe elevation in eosinophils, prompting extensive infectious (specific to her risks including overseas exposure in parts of Southeast Asia) and oncological workup to include bone marrow biopsy to rule out causes of PBE. Without another clear cause for her lab abnormality, oral vancomycin was restarted with immediate resolution of the PBE. She was continued on vancomycin for 14 months with no recurrence to date.

Discussion: This case describes a phenomenon of vancomycin-responsive PBE that was not explained by infectious, oncologic, or drug-induced causes in a patient diagnosed with UC and overlap syndrome. While oral vancomycin has been shown to improve cholangitis with normalization of liver enzymes a nd inflammatory markers, resolution of PBE has not been previously described. Exact mechanism of this process is unknown, and longitudinal monitoring of this patient will help elucidate the effects of oral vancomycin on PBE.

Conclusion: The PBE onset and resolution correlated with weaning of vancomycin and reestablishment of long-term vancomycin therapy, respectively. This phenomenon between PBE and oral vancomycin has not been previously described to our knowledge. Direct causation or exact mechanism are unknown, and longitudinal monitoring of this patient and other similar case reports will help to elucidate the effects of oral vancomycin on PBE. General pediatricians and pediatric subspecialists in the military need to continue to have a high index of suspicion for IBD and extraintestinal manifestations of IBD. Military families with known IBD should have access to long-term specialist care to provide adequate care.