38 research outputs found

    Factors associated with response to therapy and outcome of patients with primary biliary cirrhosis with features of autoimmune hepatitis

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    Background & Aims: For patients with primary biliary cirrhosis (PBC) with features of autoimmune hepatitis (AIH), treatment with ursodeoxycholic acid (UDCA) alone or in combination with immunosuppression is controversial. Little is known about the factors associated with initial response to therapy or outcome. We performed a retrospective analysis of treatment strategies and factors associated with outcomes of patients with PBC-AIH. Methods: We analyzed data from 88 patients who were diagnosed with PBC-AIH according to Paris criteria, from 7 centers in 5 countries. First-line therapies included UDCA alone (n= 30) or a combination of UDCA and immunosuppression (n= 58). Results: Of patients who received UDCA alone as the first-line therapy, 37% did not respond to treatment. Severe interface hepatitis was independently associated with lack of response to treatment (P= .024; odds ratio, 0.05; 95% confidence interval, 0.004-0.68). The combination of UDCA and immunosuppression was effective in 73% of patients who had not been previously treated or had not responded to UDCA. The presence of advanced fibrosis was associated with lack of response to the combination of UDCA and immunosuppression (P= .003; odds ratio, 0.13; 95% confidence interval, 0.03-0.48). Second-line immunosuppressive agents (cyclosporine, tacrolimus, and mycophenolate mofetil) led to biochemical remission in 54% of patients who did not respond to initial immunosuppression. Liver transplants were given to 4patients with PBC-AIH. Five patients died during follow-up (3 from liver-related causes). Conclusions: In a retrospective study of a large cohort of patients with PBC-AIH, UDCA alone did not produce a biochemical response in most patients with severe interface hepatitis; these patients require additional therapy with immunosuppression. Second-line immunosuppressive agents are effective in controlling disease activity in patients who do not respond to conventional immunosuppression. © 2014 AGA Institute

    Liver fibrosis may reduce the efficacy of budesonide in the treatment of autoimmune hepatitis and overlap syndrome.

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    BACKGROUND AND AIM: The aim of the present study was to assess the efficacy and tolerability of budesonide as an alternative first line treatment option for autoimmune hepatitis (AIH) and the overlap syndrome. METHODS: A total of 18 AIH or overlap syndrome patients were evaluated. Outcomes of treatment by the end of the study were defined as treatment failure, partial response, complete response and remission. RESULTS: Complete response and remission were achieved in 61.1% (11/18) of patients, while 38.9% (7/18) of patients were considered treatment failures. Liver fibrosis was observed in 55.5% of patients' biopsies. More patients with liver fibrosis failed to respond to treatment compared to patients without fibrosis, a difference bordering on statistical significance (60% vs. 12.5%; p=0.066). Although statistically insignificant, the presence of at least one side effect was observed more frequently in patients with fibrosis compared to those without fibrosis (80% vs. 37.5%; p=0.145). Overall, side effects occurred significantly more commonly in non-responders than responders (100% vs. 36%; p=0.013). CONCLUSIONS: Budesonide is an effective treatment option for the management of AIH, with a low incidence of side effects in patients without findings of advanced liver disease. The presence of liver fibrosis may increase the likelihood of treatment failure as well as the risk of developing side effects. Our study findings suggest that budesonide may be effective in a select group of AIH patients. Further studies are needed to determine its exact place for the treatment of AIH and overlap syndrome
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