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Autoimmun Rev. 2014 Apr-May;13(4-5):441-4. doi: 10.1016/j.autrev.2014.01.041. Epub 2014 Jan 11.

The diagnosis of primary biliary cirrhosis.

Author information

1
Division of Gastroenterology and Hepatology, University of California Davis, United States; Division of Rheumatology, Allergy and Clinical Immunology, University of California Davis, United States. Electronic address: clbowlus@ucdavis.edu.
2
Division of Gastroenterology and Hepatology, University of California Davis, United States; Division of Rheumatology, Allergy and Clinical Immunology, University of California Davis, United States.

Abstract

Primary biliary cirrhosis (PBC) is a chronic liver disease characterized by the immune mediated destruction of small intrahepatic bile duct epithelial cells leading to cholestasis and cirrhosis. The autoimmune basis of PBC is supported by the highly specific anti-mitochondrial antibodies (AMAs) and autoreactive T cells, the former being the basis for diagnosis in the vast majority of cases. Although a rare disease, the incidence rates of PBC have been increasing, possibly due to increased testing and diagnosis as opposed to a true increase in disease incidence. Presently, most cases are asymptomatic and only suspected based upon routine liver tests. Those with symptoms typically complain of pruritus and fatigue. The diagnosis of PBC is based on the presence of at least 2 of 3 key criteria including a persistently elevated serum alkaline phosphatase, the presence of serum AMAs, and liver histology consistent with PBC. Anti-nuclear antibodies specific to PBC are useful in cases in which AMAs are not detected and may indicate a more aggressive course. Ursodeoxycholic acid is the only proven therapy for PBC and in most cases can delay or prevent disease progression. However, a subgroup of patients does not adequately respond to ursodeoxycholic acid and for whom new therapies are needed.

KEYWORDS:

Anti-mitochondrial antibody; Anti-nuclear antibody; Diagnosis; Epidemiology; Primary biliary cirrhosis

PMID:
24424173
PMCID:
PMC3987745
DOI:
10.1016/j.autrev.2014.01.041
[Indexed for MEDLINE]
Free PMC Article
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