Sclerosing cholangitis can be divided into primary sclerosing cholangitis( PSC) and secondary sclerosing cholangitis according to the etiology and pathogenesis,and the pathogenesis of PSC may be associated with immunity. In recent years,a new type of sclerosing cholangitis has been found,which is characteristic of elevated levels of Ig G4 in serum and tissue and good response to steroid therapy. PSC and Ig G4- related sclerosing cholangitis can be collectively referred to as autoimmune sclerosing cholangitis. This article explores the similarities and differences between various diseases with manifestations of sclerosing cholangitis in the new concept,which will provide new ideas and directions for immune- related diseases.