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Autoimmune hepatitis overlap syndromes: an evaluation of treatment response, long‐term outcome and survival
Author(s) -
ALCHALABI T.,
PORTMANN B. C.,
BERNAL W.,
MCFARLANE I. G.,
HENEGHAN M. A.
Publication year - 2008
Publication title -
alimentary pharmacology and therapeutics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.308
H-Index - 177
eISSN - 1365-2036
pISSN - 0269-2813
DOI - 10.1111/j.1365-2036.2008.03722.x
Subject(s) - autoimmune hepatitis , medicine , overlap syndrome , gastroenterology , primary biliary cirrhosis , primary sclerosing cholangitis , hepatitis , disease
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.