Type II Autoimmune Hepatitis and Small Duct Sclerosing Cholangitis in a Seven Years Old Child: An Overlap Syndrome?

Hepatitis Monthly
Andrea Domenico PraticoSalvatore Leonardi

Abstract

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). 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. 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.

References

Mar 7, 2001·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·G V GregorioG Mieli-Vergani
May 25, 2002·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Paul AnguloKeith D Lindor
Nov 26, 2002·Seminars in Liver Disease·Albert J Czaja
May 16, 2006·Arquivos De Gastroenterologia·Maria Angela Bellomo-BrandãoGabriel Hessel
Jun 10, 2008·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Elke M HennesUNKNOWN International Autoimmune Hepatitis Group
Jun 30, 2009·Journal of Pediatric Gastroenterology and Nutrition·Giorgina Mieli-VerganiKaren F Murray
Nov 12, 2010·Journal of Hepatology·Kirsten Muri BobergUNKNOWN International Autoimmune Hepatitis Group
Nov 29, 2011·Best Practice & Research. Clinical Gastroenterology·Giorgina Mieli-Vergani, Diego Vergani
Oct 25, 2012·Digestive Diseases·Christian P Strassburg
Oct 23, 2012·Digestive Diseases and Sciences·Marilena DurazzoSimona Bo

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Methods Mentioned

BETA
ESR
biopsy
SMA

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