Autoimmune hepatitis overlap syndromes: an evaluation of treatment response, long-term outcome and survival.
Aliment Pharmacol Ther
; 28(2): 209-20, 2008 Jul.
Article
em En
| MEDLINE
| ID: mdl-18433467
ABSTRACT
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.
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Base de dados:
MEDLINE
Assunto principal:
Ácido Ursodesoxicólico
/
Colagogos e Coleréticos
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Colangite Esclerosante
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Hepatite Autoimune
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Cirrose Hepática Biliar
Idioma:
En
Ano de publicação:
2008
Tipo de documento:
Article