Long-term management and prognosis of autoimmune hepatitis (AIH): a single center experience.
BACKGROUND
Controlled trials have firmly established the need for immunosuppressive therapy in autoimmune hepatitis. However, reports about long-term management and prognosis of the disease are scarce.


METHODS
We reviewed the charts of 103 consecutive patients with a well-documented long-term course of autoimmune hepatitis who had been carefully managed over a mean observation period of 95 months (12-405 months).


RESULTS
Under immunosuppressive therapy 94 patients (91.2%) reached complete remission after a mean treatment duration of 3 +/- 3 months. 28 of the 103 patients (27.2%) were eligible for a trial of treatment withdrawal after a mean treatment duration of 32.2 months (range: 12-81 months). 21 of these patients (75%) had a relapse following treatment withdrawal. 13.6% of patients had intolerance of or severe side effects to azathioprine. There was no increase in tumor risk during a cumulative observation period of 423 patient-years of azathioprine therapy. Corticosteroid side effects occurred mostly during induction therapy, but were usually minor and resolved upon dose reduction. During a cumulative observation period of 842 patient-years no liver related deaths occurred and no patient had to be referred to liver transplantation, even though 30 patients (29.1%) had histological evidence of cirrhosis at presentation. The overall 5- and 10-year survival of patients with autoimmune hepatitis was identical to an age- and sex-matched control population.


CONCLUSIONS
Our study shows that the majority of patients with AIH do achieve a complete remission within 3 months, but require long-term or permanent immunosuppressive therapy that is usually well tolerated. Long-term survival in well-managed patients is excellent.
Kanzler
S
S
I. Department of Medicine, Johannes Gutenberg University, Mainz, Germany.
Löhr
H
H
Gerken
G
G
Galle
P R
PR
Lohse
A W
AW
D000293:Adolescent; D000328:Adult; D000368:Aged; D001379:Azathioprine / Q000008:administration & dosage / Q000009:adverse effects; D001706:Biopsy; D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000009:adverse effects; D004359:Drug Therapy, Combination; D005260:Female; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D008099:Liver / Q000473:pathology; D008111:Liver Function Tests; D008134:Long-Term Care; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage / Q000009:adverse effects; D011379:Prognosis; D012189:Retrospective Studies
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; D001379:Azathioprine