Fibrosing Cholestatic Hepatitis C in a Patient with Systemic Lupus Erythematosus

Abstract

Fibrosing cholestatic hepatitis (FCH) is a rare form of liver injury characterized by aggresssive clinical course and a propensity to liver failure. Almost all reported cases of FCH were associated with solid organ transplantation. We report a rare non-transplant patient with systemic lupus erythematosus and hepatitis C virus (HCV) infection, who developed FCH. The diagnosis was confirmed by histopathological examinations of the liver obtained at biopsy and autopsy, which were typical of FCH, including dense fibrosis, marked hepatocanalicular cholestasis, ballooning of the hepatocytes, and relatively mild inflammation. In cases of FCH, extremely high viral loads were reported under excessive immunosuppressive status. Direct cytopathic effects of the HCV have been proposed to trigger the development of FCH. The patient was infected with HCV genotype 1a and had a moderate viral load. However, immunohistochemical staining revealed abundant deposition of HCV core protein around the periportal hepatocytes, where histological changes of FCH were noted. Immunohistochemical staining also showed activated hepatic stellate cells, suggesting underlying profibrogenic processes.