The aetiology of primary sclerosing cholangitis (PSC) is not known and controversy exists as to whether PSC should be denominated an autoimmune disease. A large number of autoantibodies have been detected in PSC patients,but the specifi city of these antibodies is generally low,and the frequencies vary largely between different studies. The presence of autoantibodies in PSC may be the result of a nonspecifi c dysregulation of the immune system,but the literature in PSC points to the possible presence of specifi c antibody targets in the biliary epithelium and in neutrophil granulocytes. The present review aims to give an overview of the studies of autoantibodies in PSC,with a particular emphasis on the prevalence,clinical relevance and possible pathogenetic importance of each individual marker. The large number of autoantibodies have been detected in PSC patients, but the specifi city of these antibodies is generally low, and the results vary substantially among different studies. The presence of autoantibodies in PSC may be the result of a nonspecifi c dysregulation of the immune system, but the literature in PSC points to the possible presence of specifi c antibody targets in the biliary epithelium and in neutrophil The present review aims to give an overview of the studies of autoantibodies in PSC, with a particular emphasis on the prevalence, clinical relevance and possible pathogenetic importance of each individual marker.