31 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

    Autoimmune hepatitis/primary biliary cirrhosis overlap syndrome and associated extrahepatic autoimmune diseases.

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    To assess the prevalence of concurrent extrahepatic autoimmune diseases in patients with autoimmune hepatitis (AIH)/primary biliary cirrhosis (PBC) overlap syndrome and applicability of the 'mosaic of autoimmunity' in these patients.The medical data of 71 AIH/PBC overlap patients were evaluated for associated autoimmune diseases.In the study population, 31 (43.6\%) patients had extrahepatic autoimmune diseases, including autoimmune thyroid diseases (13 patients, 18.3\%), Sj\uf6gren syndrome (six patients, 8.4\%), celiac disease (three patients, 4.2\%), psoriasis (three patients, 4.2\%), rheumatoid arthritis (three patients, 4.2\%), vitiligo (two patients, 2.8\%), and systemic lupus erythematosus (two patients, 2.8\%). Autoimmune hemolytic anemia, antiphospholipid syndrome, multiple sclerosis, membranous glomerulonephritis, sarcoidosis, systemic sclerosis, and temporal arteritis were identified in one patient each (1.4\%). A total of 181 autoimmune disease diagnoses were found in our patients. Among them, 40 patients (56.4\%) had two, 23 (32.3\%) had three, and eight (11.3\%) had four diagnosed autoimmune diseases.A large number of autoimmune diseases were associated with AIH/PBC overlap patients. Therefore, extended screening for existing autoimmune diseases during the routine assessment of these patients is recommended. Our study suggests that the concept of 'mosaic of autoimmunity' is a valid clinical entity that is applicable to patients with AIH/PBC overlap syndrome

    Sequential presentation of primary biliary cirrhosis and autoimmune hepatitis.

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    Primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome is used to describe the coexistence of both diseases, with either a sequential or a simultaneous presentation in the same patient. Available studies have focused on the simultaneous form, whereas there is limited information on sequential PBC-AIH. We carried out a retrospective study of patients who sequentially developed PBC-AIH overlap syndrome.The medical data of 1065 patients diagnosed with PBC (n=483) and AIH (n=582) were retrospectively analyzed.A sequential development of PBC-AIH was observed in 19 (1.8\%) patients after a mean of 6.5 (1-14) years of follow-up. AIH developed in 12 (2.5\%) PBC patients, whereas PBC occurred in seven (1.2\%) patients with AIH. The baseline serologic and histological findings of patients who developed PBC-AIH were similar to those of patients with typical PBC or AIH. Eighteen patients were treated with a combination of ursodeoxycholic acid (UDCA) and immunosuppression after the diagnosis of PBC-AIH was established. One patient showed a spontaneous resolution of hepatitic flare under UDCA therapy. Biochemical remission was achieved in 16 patients, whereas three progressed to decompensated cirrhosis and required liver transplantation.The sequential overlap of PBC-AIH can occur during the follow-up of patients with pure PBC or AIH. In our cohort, we could not identify any factors that predicted the development of this rare condition. The combination of UDCA and immunosuppression seems to be an appropriate therapy in the setting of PBC-AIH
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