Features and outcome of autoimmune hepatitis type 2 presenting with isolated positivity for anti-liver cytosol antibody

Clin Gastroenterol Hepatol. 2004 Sep;2(9):825-30. doi: 10.1016/s1542-3565(04)00354-4.

Abstract

Background and aims: Autoimmune hepatitis (AIH) type 2 is identified by the presence in the serum of anti-liver/kidney microsome type 1 autoantibody. Anti-liver cytosol autoantibody has been reported in children with autoimmune liver disorders mostly in association with anti-liver/kidney microsome reactivity. However, its role as a sole marker of AIH type 2 is debated. We describe here a series of 18 children and adolescents (15 girls, 3 boys) with AIH with serum anti-liver cytosol type 1 (aLC1) as the only autoimmune marker.

Methods: A retrospective review was conducted from 3 pediatric hepatology units of all children with an autoimmune liver disease associated with aLC1 as found by immunofluorescence and/or immunodiffusion or immunoblotting.

Results: Age at first symptoms ranged from 11 months to 14 years; 12 children presented with acute hepatitis, 1 with progressive jaundice, and 5 were asymptomatic. Anti-liver/kidney microsome, antimitochondria, and anti-actin autoantibodies were not detected. Signs of cirrhosis were present in 11 children. Immunosuppressive treatment was effective in all except 2 children who had subfulminant hepatic failure and who required liver transplantation. Sixteen patients (14 with their native liver) currently are alive; 14 patients still are on immunosuppressive therapy after 1 to 22 years. According to the international scoring system for the diagnosis of AIH, 16 patients corresponded to a definite diagnosis and 2 corresponded to a probable diagnosis.

Conclusions: The presence of aLC1 in children with acute or chronic liver disease of unknown origin strongly supports a diagnosis of AIH and is an indication for early immunosuppressive therapy.

MeSH terms

  • Adolescent
  • Autoantibodies / blood*
  • Biomarkers / blood
  • Child
  • Child, Preschool
  • Female
  • Fluorescent Antibody Technique
  • Glucocorticoids / therapeutic use
  • Hepatitis, Autoimmune / diagnosis*
  • Hepatitis, Autoimmune / drug therapy
  • Hepatitis, Autoimmune / immunology
  • Humans
  • Infant
  • Male
  • Prednisone / therapeutic use
  • Prothrombin
  • Retrospective Studies

Substances

  • Autoantibodies
  • Biomarkers
  • Glucocorticoids
  • anti-liver cytosolic protein 1
  • Prothrombin
  • Prednisone