Document Detail

Autoimmune hepatitis overlap syndromes: an evaluation of treatment response, long-term outcome and survival.
MedLine Citation:
PMID:  18433467     Owner:  NLM     Status:  MEDLINE    
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.
T Al-Chalabi; B C Portmann; W Bernal; I G McFarlane; M A Heneghan
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Publication Detail:
Type:  Comparative Study; Journal Article     Date:  2008-04-23
Journal Detail:
Title:  Alimentary pharmacology & therapeutics     Volume:  28     ISSN:  1365-2036     ISO Abbreviation:  Aliment. Pharmacol. Ther.     Publication Date:  2008 Jul 
Date Detail:
Created Date:  2008-07-09     Completed Date:  2008-10-13     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8707234     Medline TA:  Aliment Pharmacol Ther     Country:  England    
Other Details:
Languages:  eng     Pagination:  209-20     Citation Subset:  IM    
Institute of Liver Studies, King's College Hospital, Denmark Hill, London, UK.
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MeSH Terms
Aged, 80 and over
Child, Preschool
Cholagogues and Choleretics / therapeutic use*
Cholangitis, Sclerosing / drug therapy*,  mortality,  pathology
Hepatitis, Autoimmune / drug therapy*,  mortality,  pathology
Liver Cirrhosis, Biliary / drug therapy*,  mortality,  pathology
Middle Aged
Ursodeoxycholic Acid / therapeutic use*
Reg. No./Substance:
0/Cholagogues and Choleretics; 128-13-2/Ursodeoxycholic Acid

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