Epidemiology and natural history of primary biliary cirrhosis in a U.S. community

Kim, Woojin; Lindor, Keith D.; Locke, G. Richard; Therneau, Terry M.; Homburger, Henry A.; Batts, Kenneth P.; Yawn, Barbara P.; Petz, Janice L.; Melton, L. Joseph; Dickson, E. Rolland

doi:10.1053/gast.2000.20197

Cited by 324 publications

(201 citation statements)

References 30 publications

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“…For example, the 95% CI for the frequency of AMA positivity in the group of ALF patients studied is 0.2895-0.5213 (P Ͻ 0.0001); this is significantly higher than the prevalence of PBC AMA, which varies from 19.1 per million to 402 per million. 22,23 Similarly, the 95% confidence limits for the frequency of AMA-positive subjects/total number of subjects at 1-7 days, 12 months, and 24 months are 0.2621-0.4908, 0.1237-0.4681, and -0.0639-0.2059, respectively (P Ͻ 0.0001).…”

Section: Resultsmentioning

confidence: 95%

Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis

et al. 2007

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In our previous work, including analysis of more than 10,000 sera from control patients and patients with a variety of liver diseases, we have demonstrated that with the use of recombinant autoantigens, antimitochondrial autoantibodies (AMAs) are only found in primary biliary cirrhosis (PBC) and that a positive AMA is virtually pathognomonic of either PBC or future development of PBC. Although the mechanisms leading to the generation of AMA are enigmatic, we have postulated that xenobiotic-induced and/or oxidative modification of mitochondrial autoantigens is a critical step leading to loss of tolerance. This thesis suggests that a severe liver oxidant injury would lead to AMA production. We analyzed 217 serum samples from 69 patients with acute liver failure (ALF) collected up to 24 months post-ALF, compared with controls, for titer and reactivity with the E2 subunits of pyruvate dehydrogenase, branched chain 2-oxo-acid dehydrogenase, and 2-oxo-glutarate dehydrogenase. AMAs were detected in 28/69 (40.6%) ALF patients with reactivity found against all of the major mitochondrial autoantigens. In addition, and as further controls, sera were analyzed for autoantibodies to gp210, Sp100, centromere, chromatin, soluble liver antigen, tissue transglutaminase, and deaminated gliadin peptides; the most frequently detected nonmitochondrial autoantibody was against tissue transglutaminase (57.1% of ALF patients). Conclusion: The strikingly high frequency of AMAs in ALF supports the thesis that oxidative stress-induced liver damage may lead to AMA induction. The rapid disappearance of AMAs in these patients provides further support for the contention that PBC pathogenesis requires additional factors, including genetic susceptibility. (HEPATOLOGY 2007;46:1436-1442

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Section: Resultsmentioning

confidence: 95%

Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis

et al. 2007

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“…1 PBC is characterized by female predominance and disease-specific serum anti-mitochondrial (AMA) and anti-nuclear (ANA) autoantibodies. 1 PBC should be considered as a rare disease based on its prevalence in the United States of 4/10,000 2 . Based on these data, less than 200,000 affected individuals should be expected in the general US population, thus fulfilling the criteria of the 2002 Rare Disease Act (HR 4013).…”

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confidence: 99%

Risk factors and comorbidities in primary biliary cirrhosis: A controlled interview-based study of 1032 patients

et al. 2005

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“…PBC is considered to be associated with disturbances in both cellular and humoral immunity, but the etiology is still uncertain. Although PBC has been described in virtually all parts of the world, 1,2 most of the epidemiologic data have been derived from Europe, 3,4 and there are not so many reports from Asia.…”

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confidence: 99%

A Cross-Sectional Study of Primary Biliary Cirrhosis in Japan: Utilization of Clinical Data When Patients Applied to Receive Public Financial Aid

Sakauchi

Mori

Zeniya

et al. 2005

Journal of Epidemiology

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BACKGROUND: There have not been many reports regarding primary biliary cirrhosis (PBC) in Asia. We conducted a cross-sectional study of PBC in Japan. METHODS: In fiscal year 1999, 9,761 patients with symptomatic PBC were registered to receive public financial aid from the Ministry of Health, Labour and Welfare of Japan. For our cross-sectional study we chose 5,805 patients whose clinical data had been written between 1999 and 2000, and statistically analyzed the data, including sex, age, major symptoms, and laboratory data. RESULTS: Our study estimated that the male-to-female ratio was 1:8.0. The median ages of male and female patients were 59 and 60 years, respectively. The major symptoms and physical findings were as follows: pruritus 53.3%, jaundice 11.3%, xanthomas 5.8%, splenomegaly 38.1%, and esophageal varices 19.1%. Antimitochondrial antibody (AMA) was positive in 86.6%, but its positive rate was lower among Japanese patients than among those in western countries. IgM levels were higher among AMA-positive patients than among AMA-negative patients. Regarding Sjögren’s syndrome, rheumatoid arthritis, chronic thyroiditis, and scleroderma, patients had lower frequencies of complicated autoimmune diseases than those in western countries. CONCLUSIONS: The male-to-female ratio, frequencies by age group, symptoms and physical findings among patients with PBC were consistent with previous reports in Japan and from other countries. However, positivity of AMA and the frequency of complicated autoimmune diseases were lower among patients in Japan than among those in western countries.

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Epidemiology and natural history of primary biliary cirrhosis in a U.S. community

Cited by 324 publications

References 30 publications

Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis

Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis

Risk factors and comorbidities in primary biliary cirrhosis: A controlled interview-based study of 1032 patients

A Cross-Sectional Study of Primary Biliary Cirrhosis in Japan: Utilization of Clinical Data When Patients Applied to Receive Public Financial Aid

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