Abstract

INTRODUCTION: Fibrosing cholestatic hepatitis (FSC) is a rare disorder, typically seen in immunosuppressed patients with a history of chronic hepatitis B and C infections. We present a case of a 67-year-old recipient of a hepatitis C virus positive donor liver with subsequent development of FSC. CASE DESCRIPTION/METHODS: We present a case of a 67 year-old gentleman, with a past medical history of HCV cirrhosis and multifocal hepatocellular carcinoma (HCC), who underwent orthotopic liver transplantation (OLT). Prior to transplantation, the patient was treated for HCV with Vosevi/ribavirin with sustained viral response. His course was complicated by multifocal HCC, for which he received treatments with DEB-TACE and Y90. He subsequently underwent OLT with a HCV positive (RNA quantitative positive) donor liver. The patient's post-operative course was complicated by marked elevation in AST and ALT (1610 and 647 respectively) and HCV RNA quantitative (1.5 million), concerning for fibrosing cholestatic hepatitis. A liver biopsy was performed and showed both mild acute cellular rejection (ACR) and cholestasis. The patient was treated with Epclusa for HCV and immunosuppression (prednisone, tacrolimus) for ACR with improvement in his liver enzymes. DISCUSSION: Fibrosing cholestatic hepatitis (FSC) is a rare disorder, typically seen in immunosuppressed patients with a history of chronic hepatitis B and C infections. It is particularly seen in patients following transplantation, namely recipients of donor liver and kidneys. Patients with FSC present with a number of symptoms including malaise, fatigue, jaundice, abdominal pain and progressive liver failure, which may occur over a period of weeks to months. The pathophysiology is related to direct cytotoxic effect of viral antigens on hepatocytes in the setting of immunosuppression. High viral titers and elevated liver enzymes in an immunosuppressed patient should raise concern for FSC. The diagnosis is established with a liver biopsy, which typically reveals hepatocyte ballooning, bridging fibrosis, pericellular and sinusoidal fibrosis as well as intracellular and canalicular cholestasis. This ultimately leads to acute liver failure and may result in rapid graft loss. Given the high rates of mortality associated with FSC, prompt diagnosis and treatment with direct-acting antiviral regimens may be effective in treatment, even in severe cases.

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