Article thumbnail

Autoimmune liver disease - are there spectra that we do not know?

By Hind I Fallatah and Hisham O Akbar

Abstract

Autoimmune liver diseases (AILDs) are common leading causes for liver cirrhosis and terminal stage of liver disease. They have variable prevalence among patients with liver disease and have two major clinical and biochemical presentations. Autoimmune hepatitis (AIH) is the typical example of hepatocellular AILD, but it can also be presented under a cholestatic pattern. AIH has a scoring diagnostic system and respond in most cases to the treatment with prednisolone and azathioprine. Primary biliary cirrhosis (PBC) is the second most common AILD, with a cholestatic presentation and characterized by positive antimitochondrial antibody (AMA). It has an excellent response and long term outcome with the administration of ursodeoxycholic acid (UDCA). Another AILD that is thought to be a variant of PBC is the autoimmune cholangitis, being a disease that has biochemical and histological features similar to PBC; but the AMA is negative. Primary sclerosing cholangitis (PSC) is a rare entity of AILD that has a cholestatic presentation and respond poorly to the treatment, with the ultimate progression to advance liver cirrhosis in most patients. Other forms of AILD include the overlap syndromes (OS), which are diseases with mixed immunological and histological patterns of two AILD; the most commonly recognized one is AIH-PBC overlap (AIH-PSC overlap is less common). The treatment of OS involves the trial of UDCA and different immunosuppressants. Here we present three case reports of unusual forms of chronic liver diseases that most likely represent AILD. The first two patients had a cholestatic picture, whereas the third one had a hepatocellular picture at presentation. We discussed their biochemical, immunological and histological features as well as their response to treatment and their outcomes. Then, we compared them with other forms of AILD

Topics: Case Report
Publisher: BioMed Central
OAI identifier: oai:pubmedcentral.nih.gov:3179434
Provided by: PubMed Central

To submit an update or takedown request for this paper, please submit an Update/Correction/Removal Request.

Suggested articles

Citations

  1. (2010). A: Autoimmune hepatitis single-center experience of clinical presentation, response to treatment and prognosis in Saudi Arabia.
  2. (2005). Allam H: Autoantibodies in chronic liver disease.
  3. (2010). Almasio PL: Clinical course and outcomes of drug-induced liver injury nimesulide as the first implicated medication. Dig Liver Dis
  4. (2010). American Association for the Study of Liver Diseases: Diagnosis and management of primary Sclerosing Cholangitis. Hepatology
  5. (2007). Autoimmune diseases of the liver autoimmune hepatitis and primary biliary cirrhosis: Unfinished business. Hepatol Res
  6. (2001). Autoimmune overlap syndromes. Hepatology
  7. (2008). Beuers U: Overlap syndromes among autoimmune liver diseases.
  8. (1996). BJ: Methotrexate hepatotoxicity in psoriatics: report of 104 patients from Nova Scotia with analysis of risks from obesity diabetes and alcohol consumption during long term follow-up.
  9. (2006). Clinical and biochemical features of autoimmune hepatitis in 36 pediatric patients. Arq Gastroenterol
  10. (2009). Current and future treatments of Autoimmune Hepatitis. Expert Rev Gastroenterol Hepatol
  11. (2002). Diagnosis and treatment of autoimmune hepatitis. Hepatology
  12. (2009). Drug-induced steatohepatitis leading to cirrhosis long-term toxicity of amiodarone use. Semin Liver Dis
  13. (2009). EJ: Primary Biliary Cirrhosis. Hepatology
  14. (2009). EJ: Review Current Status of Therapy in Autoimmune Liver Disease. Ther Adv Gastroenterol
  15. (2009). European Association for the Study of the Liver: EASL Clinical Practice Guidelines: management of cholestatic liver diseases.
  16. (2005). Färkkilä M: Budesonide combined with UDCA to improve liver histology in primary biliary cirrhosis a three year randomized trial. Hepatology
  17. (2004). FB: Diagnostic and therapeutic implications of bile duct injury in autoimmune hepatitis. Liver Int
  18. (2004). FB: True antimitochondrial antibody-negative primary biliary cirrhosis low sensitivity of the routine assays or both. Clin Exp Immunol
  19. (1996). Herruzo JA: Antimitochondrial antibody-negative chronic nonsuppurative destructive cholangitis atypical primary biliary cirrhosis or autoimmune cholangitis.
  20. (2005). Hultcrantz R: Primary sclerosing cholangitis a 5-year multicenter randomized controlled study. Gastroenterology
  21. (1999). International Autoimmune Hepatitis Group report review of criteria for diagnosis of autoimmune hepatitis.
  22. (2008). International Autoimmune Hepatitis Group: Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology
  23. (1999). IR: Autoimmune cholangitis and primary biliary cirrhosis-an autoimmune enigma. Liver
  24. (2007). Lindor KD: Overlap syndromes with autoimmune hepatitis in chronic cholestatic liver diseases. Expert Rev Gastroenterol Hepatol
  25. (2005). Lohse AW: Overlap syndromes of cholestatic liver diseases and auto-immune hepatitis. Clin Rev Allergy Immunol
  26. (2008). LX: Antimitochondrial antibodynegative primary biliary cirrhosis a subset of primary biliary cirrhosis. Liver Int
  27. (1993). Meeting Report International Autoimmune Hepatitis Group. Hepatology
  28. (2001). Mieli-Vergani G: Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood a 16-year prospective study. Hepatology
  29. (2010). MK: Diagnostic utility of IgG and IgM immunohistochemistry in autoimmune liver disease.
  30. (2009). Munoz SJ: Management of autoimmune and cholestatic liver disorders. Clin Liver Dis
  31. (2008). New insights into autoimmune liver diseases. Hepatol Res
  32. (2010). Ohira H: Overlap of primary biliary cirrhosis and autoimmune hepatitis Characteristics therapy and long term outcomes.
  33. (1998). Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome clinical features and response to therapy. Hepatology
  34. (2009). S: Update on autoimmune hepatitis. WJG
  35. (2000). SB: Autoimmune cholangitis within the spectrum of autoimmune liver disease. Hepatology
  36. (2002). SD: Characteristics of autoimmune hepatitis in Taiwan the 11 years experiences of a medical center.
  37. (2008). SD: Cholestatic jaundice as the predominant presentation in a patient with Autoimmune Hepatitis. J Chin Med Assoc
  38. (2010). SD: Gastrointestinal and liver side effects of drugs in elderly patients. Best Pract Res Clin Gastroenterol
  39. (2008). The natural history of small-sclerosing cholangitis. Gastroenterology
  40. (2007). Thiéfin G: Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis a retrospective study of 115 cases of autoimmune liver disease. Gastroenterol Clin Biol
  41. (1997). Thiel DH: A comparison of the prevalence of autoantibodies in individuals with chronic hepatitis C and those with autoimmune hepatitis the role of interferon in the development of autoimmune diseases. Hepatogastroenterology
  42. (2010). Treeprasertsuk S: Autoimmune hepatitis-PBC overlap syndrome a simplified scoring system may assist in the diagnosis.
  43. (2006). Type I autoimmune hepatitis clinical course and outcome in an Italian multicentre study. Aliment Pharmacol Ther
  44. (1997). Ursodiol for primary sclerosing cholangitis: Mayo Primary Sclerosing Cholangitis-Ursodeoxycholic Group.