Autoimmune hepatitis in Italy: the Bologna experience.
OBJECTIVE
Autoimmune hepatitis affects mainly women. It is subdivided into type 1 and type 2 according to the autoantibody profile and without immunosuppression usually evolves to cirrhosis and end-stage liver failure.


METHODS
We evaluated clinical, biochemical, immunological and genetic features and treatment response of 163 consecutive Italian patients with autoimmune hepatitis.


RESULTS
At diagnosis, type 1 autoimmune hepatitis showed more inflamed liver histology and more pronounced cholestasis, whereas type 2 was more common in children. Male and female patients shared similar clinical, biochemical and immunological features. Of 89 patients with 5-year follow-up or longer, 23 patients irrespective of presenting clinical, biochemical and immunological features achieved complete remission (normal transaminases and gammaglobulin levels) which was maintained with minimal steroid dosage; attempt at treatment withdrawal led to disease exacerbation. Complete responders had more often HLA DRB1*0401 (p = 0.011) and their risk of disease progression was lower (p < 0.0001).


CONCLUSIONS
Type 1 and type 2 autoimmune hepatitis is one and the same disease. Autoimmune hepatitis has similar features in male and female patients. HLA DRB1*0401 positive patients are more likely to achieve complete remission. Continuous low-dose steroids are necessary to maintain remission, significantly reducing the risk of disease progression.
Muratori
Paolo
P
Department of Clinical Medicine, Alma Mater Studiorum, University of Bologna, Policlinico Sant'Orsola-Malpighi, Via Massarenti, 9, 40138 Bologna, Italy.
Granito
Alessandro
A
Quarneti
Chiara
C
Ferri
Silvia
S
Menichella
Rita
R
Cassani
Fabio
F
Pappas
Georgios
G
Bianchi
Francesco B
FB
Lenzi
Marco
M
Muratori
Luigi
L
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002675:Child, Preschool; D005260:Female; D006684:HLA-DR Antigens / Q000235:genetics; D059811:HLA-DRB1 Chains; D019693:Hepatitis, Autoimmune / Q000145:classification / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007558:Italy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012727:Sex Characteristics; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome; D055815:Young Adult
D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains; C553505:HLA-DRB1*04:01 antigen; D007166:Immunosuppressive Agents; D013256:Steroids