Natural history of early primary biliary cirrhosis

Metcalf, Jane; Mitchison, H.; Palmer, Jeremy M.; Jones, David; Bassendine, Margaret F.; James, Oliver

doi:10.1016/s0140-6736(96)04410-8

Cited by 398 publications

(265 citation statements)

References 16 publications

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“…(10) All the 29 patients were previously screened in a workup for another AID. (9) Liver biopsies were performed in the majority of them, and at the screening, 24 patients had histological lesions compatible with or diagnostic of PBC, suggesting that, before the advent of any clinical or biochemical manifestations, those patients did have PBC.…”

Section: Discussionmentioning

confidence: 99%

“…5. Cumulative incidence rates of PBC at 1, 3, and 5 years were 2% (95% CI, 0-7), 7% (95% CI, [2][3][4][5][6][7][8][9][10][11][12][13][14][15], and 16% (95% CI, , respectively. Neither age, sex, AMA titer, PBC-specific ANAs, personal or familial history of AID, or baseline serum levels of bilirubin, ALP, GGT, ALT, or IgM were predictive of PBC development (Table 3).…”

Section: Clinical Outcomes Of Ama-positive Patients With Nonestablishmentioning

confidence: 99%

“…(6,7) In such a situation, longitudinal studies seem to indicate AMAs to be inevitably associated with PBC development, but these studies are rather old, all originated from a single UK center and limited area, and involve no more than 29 patients in total. (8)(9)(10) So far, no large, prospective study has been conducted to assess specifically the significance of AMAs in the absence of clinical indications of PBC. The aim of the present study was therefore to evaluate, prospectively and at a large-scale level, the prevalence, clinical characteristics, and outcomes of patients with AMAs, but nonestablished diagnosis of PBC.…”

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confidence: 99%

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Large‐scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis

et al. 2016

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The prevalence, clinical characteristics, and outcomes of patients with antimitochondrial antibodies (AMAs), but no clinical evidence of primary biliary cholangitis (PBC), are largely unknown. A prospective study of AMA incidence was conducted through a nation-wide network of 63 French immunology laboratories. Clinical data from 720 of 1,318 AMApositive patients identified in 1 year were collected. Patients were categorized as either newly diagnosed with PBC (n 5 275), previously diagnosed with PBC (n 5 216), or with nonestablished diagnosis of PBC (n 5 229). The latter group was specifically evaluated. Follow-up data were collected for up to 7 years after detection of AMAs. Prevalence of AMApositive patients without evidence of PBC was 16.1 per 100,000. These patients had the following characteristics: 78% female; median age 58 years; median AMA titer 1:160; extrahepatic autoimmune disorders 46%; normal serum alkaline phosphatases (ALP) 74%; ALP above 1.5 times the upper limit of normal 13%; and cirrhosis 6%. Compared to those newly diagnosed with PBC, the patients were slightly younger, had lower AMA titers, and lower sex-ratio imbalance. Among the patients with normal ALP and no evidence of cirrhosis, the 5-year incidence rate of PBC was 16%. Whereas no patients died from PBC, the 5-year survival rate was 75%, as compared to 90% in a control, standardized population matched for age and sex (P < 0.05). Conclusion: Nearly half of the newly detected AMAs in clinical practice does not lead to a diagnosis of PBC. PBC is unrecognized in 13% of those cases. Only 1 in 6 patients with AMAs and normal ALP will develop PBC after 5 years. The mortality of AMA-positive patients without PBC is increased irrespective of the risk of PBC development. (HEPATOLOGY 2017;65:152-163).

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Section: Discussionmentioning

confidence: 99%

Section: Clinical Outcomes Of Ama-positive Patients With Nonestablishmentioning

confidence: 99%

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confidence: 99%

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Large‐scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis

et al. 2016

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“…phases. The silent or preclinical phase is characterized by isolated AMA positivity and normal serum biochemistries 11 ; this phase may last many years. The next phase is characterized by gradual elevation of the serum ALP levels.…”

Section: Introductionmentioning

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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“…1,2 Although AMAs are found in more than 90% of patients with PBC, there has not been a specific pathogenic role defined for these autoantibodies, which often are detectable for several years before the onset of clinical disease. 3 Similarly, although infiltrating T cells have been found in the small bile ducts of PBC, the mechanisms involved in the destruction of biliary epithelial cells remain enigmatic. 4 Notwithstanding this uncertainty, the immune response in PBC and the classification of effector cells and molecules associated with disease strongly suggests that PBC is a mucosal disease.…”

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confidence: 99%

Caspase induction by IgA antimitochondrial antibody: IgA-mediated biliary injury in primary biliary cirrhosis

et al. 2004

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Natural history of early primary biliary cirrhosis

Cited by 398 publications

References 16 publications

Large‐scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis

Large‐scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis

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

Caspase induction by IgA antimitochondrial antibody: IgA-mediated biliary injury in primary biliary cirrhosis

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