Abstract

SAO SIMAO, Teresa et al. Primary sclerosing cholangitis overlapping with autoimmune hepatitis: a case with sequentially presentation over the years. Nascer e Crescer [online]. 2012, vol.21, n.2, pp.102-106. ISSN 0872-0754.

Introduction: Within the spectrum of autoimmune liver disease some patients present with features of both cholestatic disease (primary sclerosing cholangitis of large or small ducts, or primary biliary cirrhosis, the latter not described in children) and autoimmune hepatitis. These situations are difficult to classify and are called overlap syndromes. The characteristics of the overlap include symptoms, findings on physical examination, biochemical tests, immunological abnormalities, and / or histology. There are several types of associations between the various autoimmune liver diseases affecting the same patient. Case report: The authors describe the case of a child with sequential presentation of primary sclerosing cholangitis of small ducts without favourable response to immunosuppressive therapy (azatioprine + prednisolone) combined with ursodeoxycholic acid, followed some years later of characteristic features of autoimmune hepatitis with favourable biochemical response to the same therapy. Comments: This case emphasizes the need of careful long term follow up of these patients to validate the diagnosis and plan the adequate management.

Keywords : autoimmune liver disease; overlap syndrome; primary sclerosing cholangitis; autoimmune hepatitis; paediatric age; ursodeoxycholic acid.

        · abstract in Portuguese     · text in Portuguese     · Portuguese ( pdf )