Patterns of autoimmunity in primary biliary cirrhosis patients and their families: a population-based cohort study

Background: Primary biliary cirrhosis (PBC) is a chronic liver disease with autoimmune features but uncertain aetiology. Increased risk of PBC among relatives of patients may reflect common environmental factors, or inherited immunogenetic susceptibility. Associations between PBC and other autoimmun...

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Bibliographic Details
Published in:QJM : An International Journal of Medicine 2004-07, Vol.97 (7), p.397-406
Main Authors: Watt, F.E., James, O.F.W., Jones, D.E.J.
Format: Article
Language:English
Subjects:
Adult
Aged
Autoantibodies - blood
Autoimmune Diseases - complications
Autoimmune Diseases - diagnosis
Autoimmune Diseases - genetics
Autoimmunity
Biological and medical sciences
Cohort Studies
Disease Susceptibility
Female
Gastroenterology. Liver. Pancreas. Abdomen
General aspects
Humans
Liver Cirrhosis, Biliary - complications
Liver Cirrhosis, Biliary - genetics
Liver Cirrhosis, Biliary - immunology
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Male
Medical sciences
Middle Aged
Other diseases. Semiology
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Summary:Background: Primary biliary cirrhosis (PBC) is a chronic liver disease with autoimmune features but uncertain aetiology. Increased risk of PBC among relatives of patients may reflect common environmental factors, or inherited immunogenetic susceptibility. Associations between PBC and other autoimmune diseases have been reported, but their true extent and pattern is unknown. Aim: To examine the prevalence and association patterns of autoimmune disease in a representative group of PBC patients. Design: Clinical cohort study. Methods: We clinically assessed members of a geographically-based PBC patient cohort (n = 160) for the presence of additional autoimmune disease, using established specific diagnostic criteria. Results: Some 53% of patients had at least one additional autoimmune condition, and 63% had serum autoantibodies other than AMA or ANA. AMA+ patients had a significantly lower prevalence of additional autoimmunity than AMA− patients (49% vs. 79%; p < 0.01). The greatest relative increase in disease prevalence was for scleroderma (8% of patients). Autoimmune disease was present in 14% of first-degree relatives. Discussion: PBC patients and their families have a wide susceptibility to autoimmunity. This observation supports an autoimmune aetiology and suggests that the genetic basis of PBC is likely to be expressed, at least in part, through factors controlling immune tolerance in general.
ISSN:1460-2725
1460-2393
DOI:10.1093/qjmed/hch078