POST-INFANTILE GIANT-CELL HEPATITIS IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS AND AUTOIMMUNE HEPATITIS

In post-infancy, multinucleated giant cell hepatitis is rare. Various conditions and diseases associated with post-infantile giant cell hepa titis have been described, but the pathogenesis remains unknown. In th is paper we review the case reports of four patients (3 male, 1 female ; aged 22 to 32 years) with primary sclerosing cholangitis and autoimm une hepatitis. The follow-up ranges from five to seven years. All pati ents showed cholestasis and repeated elevation of hepatic transaminase s. Patients with viral infections, metabolic disorders and toxic influ ences were excluded. Histopathology of liver tissue in all four patien ts revealed giant cell formation with up to 20 nuclei in 20-70% of all hepatocytes. Post-infantile giant cell hepatitis was defined histopat hologically. The clinical course of all four patients markedly improve d after immunosuppressive treatment. Further improvement was observed with the addition of ursodeoxycholic acid. Follow-up liver biopsies du ring treatment showed reduced inflammation and a decreased number of g iant cells. One patient, who initially was admitted to the hospital wi th liver cirrhosis died five years later due to a sepsis. The clinical course of the other three patients remained stable during the observa tion period, and no progression of liver fibrosis was recorded as long as immunosuppressive treatment was continued. Cholestasis and autoimm unity seem to be two important mechanisms triggering hepatic giant cel l formation in post-infancy. In the reported cases long-lasting choles tasis in primary sclerosing cholangitis together with features of auto immune hepatitis seem to have triggered the formation of syncytial hep atic giant cells. (C) Munksgaard, 1996.