Therapy response and outcome of overlap syndromes: autoimmune hepatitis and primary biliary cirrhosis compared to autoimmune hepatitis and autoimmune cholangitis.

BACKGROUND/AIMS: We have assessed two different overlap syndrome groups in patients with AIH-PBC and AIH-AIC, with respect to therapy response and outcome.

METHODOLOGY

In this retrospective, non-randomized study, a total of 22 overlap cases were collected, 12 of those had a simultaneous form of AIH-PBC and 10 of those with AIH-AIC. Two groups were compared in terms of clinical, biochemical, immunological, histological features and response to treatment. The mean follow-up time was 31.7 +/- 11.0 mo in AIH-PBC and 41.1 +/- 29.6 mo in AIH-AIC, respectively.

RESULTS

The clinical and laboratory characteristics at presentation were not significantly different between the two groups, except a higher serum IgM level and lower AIH score in AIH-PBC group compared to AIH-AIC group (p < 0.05). First-line treatment was UDCA alone in 3 of AIH-PBC group and combination of UDCA and immunsuppressives in the remaining AIH-PBC (n = 9) and in all of the AIH-AIC (n = 10). During follow-up, only one of 10 patients in IIH-AIC group, but six of 12 patients in AIH-PBC group progressed to liver failure. So, complete remission was significantly higher in the AIH-AIC than in the AIH-PBC group ( % 90 vs % 50, p = 0.045).

CONCLUSION

To our results, in cases of AIH-PBC/AIC overlap, patients with high AIH score and negative AMA should be treated with combined therapy of corticosteroids and UDCA. However, patients with low AIH score and positive AMA should use UDCA firstly, if no response, the addition of corticosteroids should be considered with close monitoring. In this cohort, the prognosis of AIH-PBC overlap was much worse than that of AIH-AIC.