title: Type II Autoimmune Hepatitis and Small Duct Sclerosing Cholangitis in a Seven Years Old Child: An Overlap Syndrome?
abstract: BACKGROUND
  Autoimmune hepatitis is an inflammatory disease with multifactorial ethiopatogenesis, characterized by lympho-monocytic infiltration of liver, presence of serum autoantibodies (ANA, SMA, LKM-1) and high levels of immunoglobulins. Overlap syndromes are defined as the association of autoimmune hepatitis with cholestatic diseases such as primary biliary cirrhosis and primary sclerosing cholangitis. The boundaries of these syndromes as distinct pathological entities are still matter of debate and they could be part of a major liver autoimmune disease. Furthermore, cholestatic diseases may present even with atypical features (AMA-negative primary cirrohosis, primary sclerosing cholangitis with normal cholangiography).
  
  
  METHODS
  We herein describe a case of a 7 year-old child affected by an overlap syndrome between type 2 autoimmune hepatitis and small duct primary sclerosing cholangitis. Although characterized by a severe onset, the disease showed a good response to treatment with prednisone and azathioprine.
  
  
  CONCLUSIONS
  The association of type 2 autoimmune hepatitis and small duct primary cholangitis has been rarely reported in literature and this report adds new data on this still unclear entity.
authors.affiliation: Department of Pediatrics, University of Catania, Catania, Italy.
authors.forename: Andrea Domenico
authors.initials: AD
authors.lastname: Pratico
authors.affiliation: Unit of Pediatrics, Lentini Hospital, Lentini, Italy.
authors.forename: Stefania
authors.initials: S
authors.lastname: Salafia
authors.affiliation: Department of Pediatrics, University of Catania, Catania, Italy.
authors.forename: Patrizia
authors.initials: P
authors.lastname: Barone
authors.affiliation: Department of Pediatrics, University of Catania, Catania, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: La Rosa
authors.affiliation: Department of Pediatrics, University of Catania, Catania, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Leonardi
keywords: Anti-Liver Kidney Microsome Antibody; Hepatitis, Autoimmune; Liver Diseases; Primary sclerosing cholangitis