Characteristics and outcome of 544 patients with autoimmune hepatitis (AIH) – a single centre experience

Aim:

The aim of this retrospective single center study was to investigate characteristics and long-term outcome in patients with histologically proven autoimmune hepatitis (AIH) during an observation period of 16 years (from 07/2002 till 06/2018).

Patients and Methods:

A total of 544 patients were included whereas 407 were female (74,8%) and 137 were male (25,2%). Median age at diagnosis was 44,7 years. In most cases, type I AIH was present (531 patients; 97,6%) as compared to patients with type II AIH (13 patients; 2,4%). An overlap to primary biliary cholangitis (PBC) was present in 89 patients (16,4%) and to primary sclerosing cholangitis (PSC) in 22 patients (4,0%). ANA-titer was positive in 422 patients (77,6%), SMA-titer was positive in 91 patients (16,7%), and finally, AMA-titer was positive in 46 patients (8,7%).

Results:

All patients were initially treated with steroids and steroid therapy was continued to maintain remission in 97,4% (529 patients). Immunosuppression with azathioprine was preferentially used in 381 patients (70,0%). However, due to intolerance or toxic hepatitis, therapy with azathioprine had to be stopped in 107 patients (19,7%). Patients were switched to cyclosporine A (CsA), tacrolimus (FK 506) or mycophenolate mofetil (MMF), respectively. Remission of underlying AIH within the first year of diagnosis could be achieved in 277 patients (50,9%). Unfortunately, during our observation period of 16 years, a total of 168 patients (30,9%) developed liver cirrhosis with portal hypertension. Evaluation for liver transplantation was necessary in 72 patients (13,2%) and – in the further course – 51 patients had to undergo this procedure. Finally, 45 patients (8,3%) of our cohort died due to liver-related complications.

Conclusion:

This is the first long-term observational study in a German cohort with a significant number of patients diagnosed with AIH. Compared to other common liver diseases as viral or alcoholic hepatitis, patients diagnosed with autoimmune liver diseases have a benign course under sufficient immunosuppressive therapy with credible complications.