High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis

Background/Aims: Traditionally, autoimmune hepatitis (AIH) and primary scle rosing cholangitis (PSC) are regarded as separate disease entities. We repo rt on a group of patients that suggests the existence of an overlap syndrom e of the two conditions and on the prevalence of this syndrome among patien ts with PSC. Furthermore, the impact of the recently revised AIH scoring sy stem for diagnosing AIH in this context was assessed. Methods: Retrospective analysis of consecutive patients of a tertiary refer ral centre for liver disease with a diagnosis of PSC. Results: Diagnosis of the overlap syndrome was established for nine patient s (8%) of a total group of 113 PSC patients. Four patients initially presen ted with features of AIH and in five cases PSC was diagnosed first. All pat ients responded to immunosuppressive therapy; in three cases long-term remi ssion was achieved. Three patients underwent liver transplantation after 4 months and 7 and 9 years, respectively. The original and revised versions o f the AIH scoring system gave essentially the same results in the patients with the PSC-AIH overlap syndrome. Conclusions: Patients with overlapping features of AIH and PSC may be more common than is currently assumed. Recognition of this syndrome is of clinic al significance, considering the important therapeutical consequences.