Primary sclerosing cholangitis can present with acute liver failure: Report of two cases

The aim of the present study was to determine whether PSC can present with acute liver failure (ALF) and to determine its frequency. Medical records from all patients with a well-defined PSC ( n=246), treated at Karolinska University Hospital, Huddinge between 1984 and 2004 were scrutinized. Information on PSC and inflammatory bowel disease (IBD) characteristics including mode of presentation of PSC was evaluated. A group of patients with ALF of indeterminate cause at our hospital diagnosed (1993–2003) was identified as a reference group ( n=46). Two patients with PSC presented with ALF (1%) and are described in detail. Both of them had an underlying IBD. Nobody in the reference group had IBD. In conclusion, PSC patients can present with ALF in approximately 1% of all patients. PSC should be considered as a differential diagnosis in patients with ALF of indeterminate cause, especially in patients with IBD.