Abstract

Purpose: Fibrosing cholestatic hepatitis is a rare, aggressive, and usually fatal form of viral hepatitis found in immunosuppressed patients. It has been reported in solid organ and bone marrow transplant recipients. We report a case of acute hepatitis C related FCH in a post-lung transplant patient. Methods: A 72 year old man who underwent a right lung transplantation nine months prior, came to the emergency department with jaundice. He reported 3–4 weeks of fatigue and one week of jaundice and tea-colored urine. He noted pruritis and easy bruising. Past medical history included COPD status post a single-right lung transplantation. His post-transplant course was complicated by CMV treated with valgancyclovir, and one episode of acute cellular rejection treated with solumedrol. His medications included tacrolimus, mycophenolate sodium, prednisone, bactrim, pravastatin, and valganciclovir. Results: His examination revealed a thin, frail man with normal vital signs, scleral icterus, jaundice, and multiple ecchymoses. He had no asterixis or spider angiomata. His jugular venous pulse was not elevated. His mental status was normal. Admission labs showed a bilirubin of 16.1 mg/dL, alkaline phosphatase 442 U/L, AST 175 U/L, and ALT 115 U/L. Serologies including HAV, HBV, HCV, and EBV were normal. CMV PCR showed undetectable virus. Contrasted CT showed ascites without vascular obstruction and no biliary ductal dilation. Liver biopsy showed a mild mixed portal infiltration consisting of lymphocytes, neutrophils, and rare plasma cells. Bile ducts showed mild distortion. There was focal interface hepatitis and mild bile ductular proliferation. Mild lobulitis was present with scattered hepatocyte apoptosis. Multiple foci of bile plugs, intracanalicular and hepatocyte cholestasis, perivenular fibrosis and hepatic venular dilation were noted. These findings were consistent with acute hepatitis C with a suggestion of fibrosing cholestatic hepatitis. All hepatotoxic medications were stopped and immunosuppression was minimized. His condition continued to deteriorate. He developed hepatic encephalopathy and progressive jaundice despite the administration of lactulose and aggressive supportive care. On day 13 of his hospitalization Hepatitis C quantitative PCR revealed over 5,000,000 copies/mL. His clinical status continued to worsen and he died after being transitioned to hospice care. Conclusion: Acute hepatitis C and fibrosing cholestatic hepatitis is a rapidly progressive form of hepatic dysfunction. There is no effective treatment of FCH in immunosuppressed patients. Reduction of immunosuppressive medications is the only feasible option. This case represents a rare presentation of acute Hepatitis C and FCH in a post-lung transplant patient.

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