title: Autoimmune hepatitis 2 years after the diagnosis of primary sclerosing cholangitis: an unusual overlap syndrome in a 17-year-old adolescent.
abstract: A 15-year-old girl was admitted in April 2004 owing to fatigue and loss of appetite. Her paediatrician had found elevated serum levels for alkaline phosphatase. The endoscopic retrograde cholangiography documented typical signs of primary sclerosing cholangitis with involvement of the small and large ducts. The liver biopsy revealed extensive septal and portal fibrosis. No evidence of inflammatory bowel disease was present. She was started on ursodeoxycholic acid therapy and improved clinically. After 22 months she presented again with rising transaminase levels up to 600 U/l. The second liver biopsy was strongly suggestive for autoimmune hepatitis besides the already known features of primary sclerosing cholangitis. Elevated levels of IgG, and elevated titres for ANA and antismooth muscle antibodies (ASMA) were also found. The duct irregularities seen on re-endoscopic retrograde cholangiography were slightly regredient as compared with the first investigation. We added prednisolone and azathioprine to the ursodeoxycholic acid and the transaminase levels dropped together with clinical improvement.
authors.affiliation: Department of Internal Medicine, Diakonie-Hospital Schwaebisch Hall, Germany.
authors.forename: Tanja
authors.initials: T
authors.lastname: Mueller
authors.forename: Leonardo
authors.initials: L
authors.lastname: Bianchi
authors.forename: Markus
authors.initials: M
authors.lastname: Menges
mesh_terms: D000293:Adolescent; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D013577:Syndrome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid