S5829 A Rare Case of CMV Hepatitis Occurring in a Young Patient With Autoimmune Polyglandular Syndrome and Pediatric-Onset Autoimmune Hepatitis

Introduction: Cytomegalovirus (CMV) is a member of Herpesviridae family, often affecting children and adolescents, with a benign course. Immunosuppressed patients may have a severe course during primary infection or reactivation. Although cases of CMV hepatitis have been reported, there is only 1 case report of CMV hepatitis occurring in a patient with autoimmune hepatitis (AIH) (on azathioprine [AZA] and steroids), along with Sweet Syndrome. Here we describe a case of CMV hepatitis in a patient with pediatric-onset AIH. Case Description/Methods: A 28-year-old woman with a history of autoimmune polyglandular syndrome and AIH, diagnosed at age 4, (on AZA 150 mg/day and prednisone 5 mg/day) presented with fatigue, fever and multiple metabolic derangements, consistent with an “adrenal crisis”. Her liver function tests were elevated: peak bilirubin 4.4, aspartate aminotransferase 439, alanine aminotransferase (ALT) 427, alkaline phosphatase 623. Infectious work-up revealed a CMV DNA of 45,300 IU. Liver biopsy showed portal and lobular lymphocytic exudates as well as intranuclear inclusions of CMV hepatitis and no histologic features of active AIH or fibrosis. CMV serologies were negative, suggesting this to be a primary infection with no ocular involvement. Her toddler relative, with whom she was in close contact, was sick with a flu-like illness before onset of the patient’s symptoms and may have been the source of exposure. AZA and steroids were held and she was treated with valganciclovir. Given a rise in CMV DNA to >4 million IU and poor tolerance, her regimen was switched to maribavir. Anti-viral resistance testing was negative. After 6 weeks of therapy, CMV DNA was undetectable and LFT’s were much improved (ALT of 124). She remains on maribavir for now, along with low dose prednisone. Discussion: This is a rare case of acute CMV hepatitis in an immunocompromised host with longstanding AIH, on maintenance AZA and steroids. Her autoimmune polyglandular syndrome may have posed additional risk. CMV hepatitis is well known to occur in other immunocompromised hosts, particularly organ transplant recipients with donor derived infections. Clinicians and immunocompromised patients should be aware of the risk of community exposure. An early diagnosis is key to instituting effective treatment and achieving a good outcome. The therapeutic challenge will be navigating re-institution of her immunosuppression regimen for AIH, while at the same time monitoring closely for persistence or reactivation of her CMV hepatitis after her anti-viral therapy is completed.