Fibrosing Cholestatic Hepatitis C in a Patient with Systemic Lupus Erythematosus

Fibrosing cholestatic hepatitis (FCH) is a rare form of liver injurycharacterized by aggresssive clinical course and a propensity toliver failure. Almost all reported cases of FCH were associated withsolid organ transplantation. We report a rare non-transplant patientwith systemic lupus erythematosus and hepatitis C virus (HCV)infection, who developed FCH. The diagnosis was confirmed byhistopathological examinations of the liver obtained at biopsy andautopsy, which were typical of FCH, including dense fibrosis,marked hepatocanalicular cholestasis, ballooning of the hepatocytes,and relatively mild inflammation. In cases of FCH, extremely highviral loads were reported under excessive immunosuppressivestatus. Direct cytopathic effects of the HCV have been proposedto trigger the development of FCH. The patient was infectedwith HCV genotype 1a and had a moderate viral load. However,immunohistochemical staining revealed abundant deposition of HCVcore protein around the periportal hepatocytes, where histologicalchanges of FCH were noted. Immunohistochemical staining alsoshowed activated hepatic stellate cells, suggesting underlyingprofibrogenic processes.