title: Type 1 and type 2 autoimmune hepatitis in adults share the same clinical phenotype.
abstract: BACKGROUND
  Autoimmune hepatitis (AIH) is historically classified into type 1 and type 2 on the basis of the autoantibody profile, anti-nuclear and/or anti-smooth muscle antibodies being the serological markers of type 1 AIH, whereas anti-liver/kidney microsomal antibody type 1 and/or anti-liver cytosol antibody type 1 characterise type 2 AIH.
  
  
  OBJECTIVE
  To evaluate whether such a distinction is justified on the basis of different expression of the disease in adults.
  
  
  METHODS
  Twenty-six adult patients with type 2 AIH and 52 age- and sex-matched patients with type 1 AIH, representative of the entire cohort of adults with type 1 AIH, were compared at onset and during follow-up.
  
  
  RESULTS
  At diagnosis, median age was 26 years (range 17-53), female sex 86%, acute presentation 43%, severe liver histology 54%, cirrhosis 14%, complete response to treatment 52%, progression of the disease 17%, and median disease duration 72 months (range 12-280). HLA-DRB1*0301 was present in 26%, HLA-DRB1*0401 in 23% and HLA-DRB1*0701 in 25%. Clinical presentation, biochemical parameters, severe liver histology, genetic profile, response rate and progression of the disease were identical between type 1 and type 2 AIH.
  
  
  CONCLUSIONS
  There is not enough clinical, biochemical, histological or genetic reason to subdivide adults with autoimmune hepatitis into type 1 and type 2 on the basis of the autoantibody profile, and the term 'autoimmune hepatitis' without qualification should be preferred.
authors.affiliation: Centro per lo Studio e la Cura delle Malattie Autoimmuni del Fegato e delle Vie Biliari, Bologna, Italy.|Dipartimento di Scienze Mediche e Chirurgiche (DIMEC), Alma Mater Studiorum - Università di Bologna, Bologna, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Muratori
authors.affiliation: Centro per lo Studio e la Cura delle Malattie Autoimmuni del Fegato e delle Vie Biliari, Bologna, Italy.|Dipartimento di Scienze Mediche e Chirurgiche (DIMEC), Alma Mater Studiorum - Università di Bologna, Bologna, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Lalanne
authors.affiliation: Centro per lo Studio e la Cura delle Malattie Autoimmuni del Fegato e delle Vie Biliari, Bologna, Italy.|Dipartimento di Scienze Mediche e Chirurgiche (DIMEC), Alma Mater Studiorum - Università di Bologna, Bologna, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Fabbri
authors.affiliation: Centro per lo Studio e la Cura delle Malattie Autoimmuni del Fegato e delle Vie Biliari, Bologna, Italy.|Azienda Ospedaliero-Universitaria di Bologna, Policlinico Sant'Orsola-Malpighi, via Massarenti 9, Bologna 40138, Bologna, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Cassani
authors.affiliation: Centro per lo Studio e la Cura delle Malattie Autoimmuni del Fegato e delle Vie Biliari, Bologna, Italy.|Dipartimento di Scienze Mediche e Chirurgiche (DIMEC), Alma Mater Studiorum - Università di Bologna, Bologna, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Lenzi
authors.affiliation: Centro per lo Studio e la Cura delle Malattie Autoimmuni del Fegato e delle Vie Biliari, Bologna, Italy.|Dipartimento di Scienze Mediche e Chirurgiche (DIMEC), Alma Mater Studiorum - Università di Bologna, Bologna, Italy.
authors.forename: L
authors.initials: L
authors.lastname: Muratori
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies; D015415:Biomarkers; D018450:Disease Progression; D005260:Female; D059811:HLA-DRB1 Chains; D019693:Hepatitis, Autoimmune / Q000235:genetics* / Q000276:immunology*; D006801:Humans; D008103:Liver Cirrhosis / Q000276:immunology; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D059811:HLA-DRB1 Chains; C553502:HLA-DRB1*03:01 antigen; C553505:HLA-DRB1*04:01 antigen; C094307:anti-liver cytosolic protein 1