Original Contribution
The American Journal of Gastroenterology (2005) 100, 1516–1522; doi:10.1111/j.1572-0241.2005.41841.x
Clinical Course and Outcome of Autoimmune Hepatitis/Primary Sclerosing Cholangitis Overlap Syndrome
This work was partially supported by an University grant (ex 60%).
Annarosa Floreani MD1, Erik Rosa Rizzotto MD1, Francesco Ferrara MD1, Isabella Carderi MD1, Diego Caroli MD1, Luigi Blasone MD1 and Vincenzo Baldo MD2
- 1Department of Surgical and Gastroenterological Sciences, University of Padova, Padova, Italy;
- 2Department of Hygiene and Public Health, Institute of Hygiene, University of Padova, Padova, Italy
Correspondence: Annarosa Floreani, Department of Surgical and Gastroenterological Sciences, Via Giustiniani, 2, 35128 Padova, Italy
Received 23 November 2004; Revised 0000; Accepted 18 February 2005.
Abstract
Autoimmune hepatitis/primary sclerosing cholangitis (AIH/PSC) overlap syndrome is a relatively uncommon variant of PSC.
AIM:
To evaluate the natural history of AIH/PSC overlap syndrome compared to a group of "classical" PSC.
METHODS:
Forty-one consecutive PSC patients, with a regular follow-up of at least 2 years, were prospectively included in the study. Among these, 7 fulfilled the criteria for AIH/PSC overlap syndrome.
RESULTS:
The AIH/PSC overlap group significantly differed from the "classical" PSC group in the following parameters: mean age at presentation (21.4 
5.0 vs 32.3 10 years, p < 0.01), AST 191.0 14.8 vs 48.9 34.5 U/L, p < 0.005), ALT (357.0 26.5 vs 83.7 60.7 U/L, p < 0.005) and serum IgG (25.6 4.7 vs 12.9 6.0 mg/dl, p < 0.0001). The mean follow-up was similar in the 2 groups (93.3 65.9 vs 98.1 65.9 months respectively). Treatment included immunosuppression + ursodeoxycholic acid (UDCA) in the AIH/PSC overlap patients, and UDCA in the "classical" PSC group. Deaths were recorded only in the classical PSC group. The median survival in the latter group was 207 months (95% C.I. 87.6-326.4). The major events during the follow-up included: OLTx (1/7 vs 6/34), and neoplasms (only in the group of "classical" PSC). The new Mayo score prognostic index only increased significantly during follow-up in the "classical" PSC group (r2 0.8117, p < 0.01)
CONCLUSION:
Patients with AIH/PSC overlap syndrome seem to benefit from immunosuppression + UDCA therapy, survival is apparently better than in "classical" PSC condition.
