Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jul 7, 2015; 21(25): 7683-7708
Published online Jul 7, 2015. doi: 10.3748/wjg.v21.i25.7683
Primary biliary cirrhosis: Clinical and laboratory criteria for its diagnosis
Vasiliy Ivanovich Reshetnyak
Vasiliy Ivanovich Reshetnyak, V.A. Negovsky Research Institute of General Reanimatology, 107031 Moscow, Russia
Author contributions: Reshetnyak VI solely contributed to this paper.
Conflict-of-interest statement: The author declare no conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Vasiliy Ivanovich Reshetnyak, MD, PhD, DSc, Professor, Academic Secretary, V.A. Negovsky Research Institute of General Reanimatology, 25-2, Petrovka street, 107031 Moscow, Russian.
Telephone: +7-495-6946505 Fax: +7-495-6946505
Received: February 4, 2015
Peer-review started: February 6, 2015
First decision: March 26, 2015
Revised: April 7, 2015
Accepted: June 10, 2015
Article in press: June 10, 2015
Published online: July 7, 2015

Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic granulomatous, and destructive inflammatory lesion of small intralobular and septal bile ducts, which is likely to be caused by an autoimmune mechanism with a the presence of serum antimitochondrial antibodies and a potential tendency to progress to cirrhosis. Despite the fact that the etiology of this disease has been unknown so far, there has been a considerable body of scientific evidence that can reveal the clinical and laboratory signs of PBC and the individual components of its pathogenesis and elaborate diagnostic criteria for the disease and its symptomatic therapy. Deficiencies in autoimmune tolerance are critical factors for the initiation and perpetuation of the disease. The purpose of this review is to summarize the data available in the literature and the author’s findings on clinical and laboratory criteria for the diagnosis of PBC. This review describes the major clinical manifestations of the disease and the mechanisms of its development. It presents the immunological, biochemical, and morphological signs of PBC and their significance for its diagnosis. A great deal of novel scientific evidence for the problem of PBC has been accumulated. However, the inadequate efficiency of therapy for the disease lends impetus to the quest for its etiological factors and to further investigations of its pathogenetic mechanisms and, on this basis, to searches for new methods for its early diagnosis.

Keywords: Primary biliary cirrhosis, Clinical criteria, Laboratory criteria, Immunological signs, Biochemical signs, Morphological signs

Core tip: Primary biliary cirrhosis is a chronic autoimmune cholestatic liver disease. This review summarizes current literature data and our own experiences on clinical and laboratory criteria for the diagnosis of primary biliary cirrhosis. Thanks to advances in biochemistry, molecular biology and genetics, it became possible to present these data with regard to the pathophysiological mechanisms of their development.