Overlap of Autoimmune Hepatitis and Primary Biliary Cirrhosis: Long-Term Outcomes

Silveira, Marina G.; Talwalkar, Jayant A.; Angulo, Paul; Lindor, Keith D.

doi:10.1111/j.1572-0241.2007.01136.x

Cited by 137 publications

(87 citation statements)

References 15 publications

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“…During follow-up, 6 of the 12 patients progressed to liver failure (including patients treated with ursodeoxycholic acid with and without immunosuppression). A comparison of 26 patients with AIH-PBC overlap syndrome and 109 PBC patients without overlap showed that patients with overlap had worse outcomes [110]. Overlap was associated with significantly higher rates of portal hypertension (P = 0.01), esophageal varices (P < 0.01), gastrointestinal bleeding (P = 0.02), ascites (P < 0.01), death and/or OLT (P < 0.05).…”

Section: Aih-pbc Overlap Syndromementioning

confidence: 93%

Autoimmune hepatitis

Sahebjam

Vierling

2015

Front. Med.

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Autoimmune hepatitis is a chronic liver disease putatively caused by loss of tolerance to hepatocyte-specific autoantigens. It is characterized by female predilection, elevated aminotransferase levels, autoantibodies, increased γ-globulin or IgG levels and biopsy evidence of interface hepatitis. It is currently divided into types 1 and 2, based on expression of autoantibodies. Autoantigenic epitopes have been identified only for the less frequent type 2. Although autoimmune hepatitis occurs in childhood, this review focuses on disease in adults. In the absence of pathognomonic biomarkers, diagnosis requires consideration of clinical, biochemical, serological and histological features, which have been codified into validated diagnostic scoring systems. Since many features also occur in other chronic liver diseases, these scoring systems aid evaluation of the differential diagnosis. New practice guidelines have redefined criteria for remission to include complete biochemical and histological normalization on immunosuppressive therapy. Immunosuppression is most often successful using prednisone or prednisolone and azathioprine; however, the combination of budesonide and azathioprine for non-cirrhotic patients offers distinct advantages. Patients failing standard immunosuppression are candidates for alternative immunosuppressive regimens, yet none of the options has been studied in a randomized, controlled trial. Overlap syndromes with either primary sclerosing cholangitis or primary biliary cirrhosis occur in a minority. Liver transplantation represents a life-saving option for patients presenting with acute liver failure, severely decompensated cirrhosis or hepatocellular carcinoma. Transplant recipients are at risk for recurrent autoimmune hepatitis in the allograft, and de novo disease may occur in patients transplanted for other indications. Patients transplanted for AIH are also at risk for recurrent or de novo inflammatory bowel disease. Progress in our understanding of the immunopathogenesis should lead to identification of specific diagnostic and prognostic biomarkers and new therapeutic strategies.

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Section: Aih-pbc Overlap Syndromementioning

confidence: 93%

Autoimmune hepatitis

Sahebjam

Vierling

2015

Front. Med.

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“…The frequency of overlap with AIH in patients with PBC varies depending on the scoring system used for diagnosis, but it is estimated to be about 10%. Patients with PBC-AIH overlap appear to have worse clinical outcomes compared with patients with PBC [27], and this observation has potential therapeutic implications.…”

Section: Pbc-autoimmune Hepatitis Overlapmentioning

confidence: 93%

Latest and Emerging Therapies for Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

Zein

Lindor

2010

Curr Gastroenterol Rep

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Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the two most common causes of chronic cholestatic liver disease in adults. In PBC, therapy with ursodeoxycholic acid (UDCA) is safe and has been associated with tangible biochemical, histologic, and survival benefits. However, a need for different or adjuvant therapies remains for specific subsets of PBC patients, including those who do not respond to UDCA and those who have advanced histologic disease at presentation. Similarly, beneficial therapies for disease-related symptoms that do not typically respond to UDCA (eg, fatigue and pruritus) are still needed. In contrast to PBC, no medical therapy of proven benefit has been identified for patients with PSC. In PBC and PSC, adequate management of complications of chronic cholestasis is important. For both diseases, liver transplantation is the only curative option.

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“…After 6 years of follow-up, the patients with overlap had significantly worse clinical outcomes than the nonoverlap PBC patients, thus confirming the potential importance of using liver biopsy to establish the correct diagnosis. 23 It was recognized that in one study, the presence of AMA and cholestatic enzymes in a middle aged woman had a 98% positive predictive value for the presence of PBC, 3 obviating the need for a liver biopsy for diagnostic purposes. However, this study was conducted and validated in centers of excellence for PBC by experienced investigators.…”

Section: Entry Criteriamentioning

confidence: 99%

“…Natural history studies have identified numerous markers of progressive disease in PBC, including but not limited to: response to UDCA, [15][16][17][18] histology (degree of interface hepatitis and features of overlap with autoimmune hepatitis), 23,24 biochemical markers (serum bilirubin, albumin and prothrombin time), 28 presence and/or degree of portal hypertension, 29 certain genetic polymorphisms (apolipoprotein A, tumor necrosis factor [TNF]-alpha), specific autoantibodies (anti-gp210, anti-promyelocytic leukemia protein [PML], anti-sp100, anti-centromere), 30,31 and serum markers of fibrosis (hyaluronic acid, procollagen III, tissue inhibitor metalloproteinase). 32 Models using time-fixed Cox proportional hazards regression analysis have been developed to predict survival in PBC.…”

Section: Stratification Of Subjectsmentioning

confidence: 99%

American association for the study of liver diseases endpoints conference: Design and endpoints for clinical trials in primary biliary cirrhosis

et al. 2010

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PreambleA group of multidisciplinary experts on primary biliary cirrhosis (PBC) and its complications convened on May 31, 2009, under the aegis of the American Association for the Study of Liver Diseases (AASLD) in order to identify the most appropriate design and endpoints for clinical trials of PBC based on current evidence and expert experience. The natural history of PBC was reviewed as well as current therapies. The current approaches to evaluating therapies for disease progression and symptoms as priorities were discussed. Appropriate aspects of trial design including entry criteria, study duration, and appropriate handling of issues such as stratification of subjects and use of ursodeoxycholic acid (UDCA), were identified and discussed. After a full day of presentations, in a consensus manner, appropriate endpoints for clinical efficacy trials regarding PBC and its complications were agreed upon and are reported in this summary.

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Overlap of Autoimmune Hepatitis and Primary Biliary Cirrhosis: Long-Term Outcomes

Cited by 137 publications

References 15 publications

Autoimmune hepatitis

Autoimmune hepatitis

Latest and Emerging Therapies for Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

American association for the study of liver diseases endpoints conference: Design and endpoints for clinical trials in primary biliary cirrhosis

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