[Autoimmune hepatitis and primary sclerosing cholangitis: dependent or independent association?].

BACKGROUND AND

OBJECTIVE

The diagnosis of an autoimmune liver disease is based on clinical, biochemical, immunological and histological criteria particular to each disease, such as autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Hepatic overlap syndromes (OS) are defined by the presence of a well established autoimmune liver disease, primarily AIH plus two or more characteristics associated with another liver disease (PBC or PSC); however the association of HAI and PSC is considered rare in adult population, and only 43 cases have been reported in the literature. The aim of this study is to review and reappraise the characteristics of this rare association, and to discuss current concepts on OS.

METHODS

We describe the clinical presentation, evolution, radiologic studies, histological characteristics and therapeutic options in a Mexican woman with OS (HAI-PSC). We also include an updated review of the literature.

RESULTS

Overlap ofAIH and PSC has been described in a number of repqrts during the last decade, and is assumed to exist in a considerable part of mainly young patients with autoimmune liver diseases. Sequential appearance of AIH and PSC has been described in children, but also may be observed in adults. This association has been reported between 1.4%-8%, probably because differences in age of the study populations, range of autoantibodies taken into consideration, and degree of completeness of analyzed data.

CONCLUSIONS

HAI-PSC is a rare disease, more common in children, and its outcome and evolution seem to be similar of AIH alone. Ursodesoxycholic acid in combination with an immunosuppressive regimen may be an adequate medical treatment for most patients with this association, and liver transplantation should be considered in late stage disease.