Autoimmune hepatitis overlap syndromes

an evaluation of treatment response, long-term outcome and survival

AL-CHALABI, T.
PORTMANN, B. C.
BERNAL, W.
MCFARLANE, I. G.
HENEGHAN, M. A.

Institute of Liver Studies, King's College Hospital, Denmark Hill, London, UK

Correspondence to: Dr M. A. Heneghan, Institute of Liver Studies, King's College Hospital, Denmark Hill, London SE5 9RS, UK. E-mail: [email protected]

Publication data Submitted 9 March 2008 First decision 31 March 2008 Resubmitted 13 April 2008 Accepted 17 April 2008 EpubOnline Accepted 22 April 2008

Alimentary Pharmacology & Therapeutics 28(2):p 209-220, July 15, 2008.

SUMMARY

Background

Primary sclerosing cholangitis/autoimmune hepatitis (PSC/AIH) and primary biliary cirrhosis/AIH (PBC/AIH) overlap syndromes are poorly defined variants of AIH. Few large patient series exist, and there are little data on long-term outcomes.

Aim

To compare presentation, clinical course and outcome of patients with PSC/AIH and PBC/AIH, with patients with definite AIH.

Methods

Two hundred and thirty-eight AIH patients were compared with 10 PBC/AIH patients and 16 PSC/AIH patients presenting consecutively between 1971 and 2005 at a single centre.

Results

Autoimmune hepatitis patients were significantly more likely to present with jaundice (69.4% vs. 25%; P = 0.0145) than PBC/AIH patients. Median serum aspartate aminotransferase activity at presentation was higher in AIH patients compared with PBC/AIH and PSC/AIH patients respectively (620 vs. 94 vs. 224 IU/L; P < 0.05). PBC/AIH patients demonstrated no response to standard AIH therapy more frequently than AIH patients (25% vs. 0.8%; P = 0.0057). Significant reduction in survival was identified between patients with PSC/AIH and those without (hazard ratio: PSC/AIH vs. AIH = 2.08, PSC/AIH vs. PBC/AIH = 2.14; P = 0.039).

Conclusions

Patients with PSC/AIH have severe disease and significantly worse prognosis than patients with AIH or PBC/AIH. Recognition and close follow-up of this cohort are warranted.