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autoimmune hepatitis type 2

Tuesday 21 February 2006

LKM1-associated autoimmune hepatitis or anti-LKM1 chronic active hepatitis

Definition: Autoimmune hepatitis (AIH) type 2 is identified by the presence in the serum of anti-liver/kidney microsome type 1 autoantibody.

Type 2 AIH, characterized by anti-liver kidney microsomal antibodies. This form is rare in adults.

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.

The autoimmune hepatitis type 2 (AIH type 2) affects predominantly pediatric patients and is characterized by a more severe clinical course, a higher frequency of relapse under immunosuppressive treatment and a more frequent progression to cirrhosis.

Type 2, which occurs in a younger age group, is associated with positive anti-liver kidney microsomal antibodies and is a more aggressive disease.

Treatment failure is more frequent and relapse after drug withdrawal is almost inevitable; most of these patients need lifelong immunosuppressive therapy.

See also

- autoimmune hepatitis

  • autoimmune hepatitis type 1


- Bridoux-Henno L, Maggiore G, Johanet C, Fabre M, Vajro P, Dommergues JP, Reinert P, Bernard O. 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. PMID: 15354284