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

Background/aims: The aim of the present study was to determine whether PSC can present with acute liver failure (ALF) and to determine its frequency.

Methods: 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).

Results: 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.

Conclusions: 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.