Clinicopathological study of primary biliary cirrhosis negative for antimitochondrial antibodies

2008 ◽  
Vol 17 (6) ◽  
pp. 281-287 ◽  
Author(s):  
Yasuni Nakanuma ◽  
Kenichi Harada ◽  
Kyousuke Kaji ◽  
Shuichi Terasaki ◽  
Koichi Tsuneyama ◽  
...  
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Biliary Cirrhosis ◽  
Antimitochondrial Antibodies ◽  
Clinicopathological Study

scholarly journals A sensitive bead assay for antimitochondrial antibodies: Chipping away at AMA-negative primary biliary cirrhosis

Hepatology ◽  
2007 ◽  
Vol 45 (3) ◽  
pp. 659-665 ◽  
Author(s):  
Sabine Oertelt ◽  
Roman Rieger ◽  
Carlo Selmi ◽  
Pietro Invernizzi ◽  
Aftab A. Ansari ◽  
...  
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Biliary Cirrhosis ◽  
Antimitochondrial Antibodies

scholarly journals Primary Biliary Cirrhosis Associated with Systemic Sclerosis: Diagnostic and Clinical Challenges

2011 ◽  
Vol 2011 ◽  
pp. 1-12 ◽  
Author(s):  
Cristina Rigamonti ◽  
Dimitrios P. Bogdanos ◽  
Maria G. Mytilinaiou ◽  
Daniel S. Smyk ◽  
Eirini I. Rigopoulou ◽  
...  
Keyword(s):  
Liver Disease ◽  
Systemic Sclerosis ◽  
General Population ◽  
Primary Biliary Cirrhosis ◽  
Early Identification ◽  
Case Reports ◽  
Biliary Cirrhosis ◽  
Antimitochondrial Antibodies ◽  
Favorable Prognosis ◽  
Limited Systemic Sclerosis

Patients with primary biliary cirrhosis (PBC) often have concurrent limited systemic sclerosis (SSc). Conversely, up to one-fourth of SSc patients are positive for PBC-specific antimitochondrial antibodies (AMA). The mechanisms responsible for the co-occurrence of these diseases are largely unknown. Genetic, epigenetic, environmental, and infectious factors appear to be important for the pathogenesis of the disease, but the hierarchy of events are not well defined. Patients with SSc and PBC have an increased morbidity and mortality compared with the general population, but whether the presence of both diseases in an affected individual worsens the prognosis and/or outcome of either disease is not clear. Some case reports suggested that the presence of SSc in PBC patents is associated with a more favorable prognosis of the liver disease, whereas others report an increased mortality in patients with PBC and SSc compared to patients with PBC alone. This paper discusses the features of patients with PBC-associated SSc. Our aims are to clarify some of the pathogenetic, diagnostic, and clinical challenges that are currently faced in the routine management of these patients. We also intend to provide some practical hints for practitioners that will assist in the early identification of patients with PBC-associated SSc.

Detection of novel non-M2-related antimitochondrial antibodies in patients with anti-M2 negative primary biliary cirrhosis

Gut ◽  
2009 ◽  
Vol 58 (7) ◽  
pp. 983-989 ◽  
Author(s):  
M Feuchtinger ◽  
S Christ ◽  
B Preuss ◽  
J Dengjel ◽  
S Duman ◽  
...  
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Biliary Cirrhosis ◽  
Antimitochondrial Antibodies

scholarly journals Characterization of Autoantibodies against the E1 Subunit of Branched-Chain 2-Oxoacid Dehydrogenase in Patients with Primary Biliary Cirrhosis

2012 ◽  
Vol 2012 ◽  
pp. 1-12 ◽  
Author(s):  
Tsutomu Mori ◽  
Hiromasa Ohira ◽  
Masahito Kuroda ◽  
Masaki Kato ◽  
Yoshiki Yamaguchi ◽  
...  
Keyword(s):  
Enzyme Activity ◽  
Primary Biliary Cirrhosis ◽  
Active Center ◽  
Epitope Mapping ◽  
Biliary Cirrhosis ◽  
Antimitochondrial Antibodies ◽  
Points Of View ◽  
Branched Chain

Primary biliary cirrhosis (PBC) is characterized by antimitochondrial antibodies (AMAs) that react with the lipoyl-containing E2 subunits of 2-oxoacid dehydrogenase complexes such as BCOADC and PDC. The lipoyl domains of E2 contain the major epitopes essential for immunopathology. However, the non-lipoyl-containing E1 subunits are also frequently targeted. Since anti-E1 antibodies always appear in combination with anti-E2 antibodies, the mechanisms underlying the autoimmunity against E1 may be linked to, but distinct from, those against E2. Here, we demonstrate that intermolecular and intramolecular determinant spreading underlies the autoimmunity against E1. We performed characterizations and epitope mapping for anti-BCOADC-E1 antibodies from both the intermolecular and intramolecular points of view. The antibody reactivities form a cluster against the BCOADC complex that is distinct from that against the PDC complex, and the anti-BCOADC-E1 antibodies arise as part of the cluster against the BCOADC complex. Multiple epitopes are present on the surface of the BCOADC-E1 molecule, and the major epitope overlaps with the active center. Sera with anti-BCOADC-E1 antibodies strongly inhibited the enzyme activity. These findings suggest that the E1 subunit as part of the native BCOADC complex is an immunogen, and that determinant spreading is involved in the pathogenesis of AMA production.

scholarly journals Rheumatoid Arthritis and Primary Biliary Cirrhosis: Cause, Consequence, or Coincidence?

Arthritis ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-7 ◽  
Author(s):  
Daniel S. Smyk ◽  
Dimitrios P. Bogdanos ◽  
Maria G. Mytilinaiou ◽  
Andrew K. Burroughs ◽  
Eirini I. Rigopoulou
Keyword(s):  
Rheumatoid Arthritis ◽  
Primary Biliary Cirrhosis ◽  
High Titre ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Infectious Agents ◽  
Antimitochondrial Antibodies ◽  
Genetic Studies ◽  
Genetic Traits ◽  
Large Case

Primary biliary cirrhosis (PBC) is a progressive cholestatic liver disease characterized serologically by cholestasis and the presence of high-titre antimitochondrial antibodies and histologically by chronic nonsuppurative cholangitis and granulomata. PBC patients often have concomitant autoimmune diseases, including arthropathies. This raises the question as to whether there are shared features in the pathogenesis of those diseases with the pathogenesis of PBC. Epidemiological and large case studies have indicated that although the incidence of rheumatoid arthritis (RA) is not significantly raised in PBC patients, there appears to be a higher rate of RA in PBC patients and their relatives. Genetic studies have demonstrated that several genes implicated in PBC have also been implicated in RA. Epigenetic studies provided a wealth of data regarding RA, but the findings on epigenetic changes in PBC are very limited. As well, certain infectious agents identified in the pathogenesis of PBC may also play a role in the pathogenesis of RA. These data suggest that although RA is not significantly present in PBC, some individuals with certain genetic traits and environmental exposures may develop both conditions. This concept may also apply to other concomitant diseases found in PBC patients.

scholarly journals Twelve-Year-Old Girl with Primary Biliary Cirrhosis

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Ivana Kitic ◽  
Aleksandra Boskovic ◽  
Ivica Stankovic ◽  
Dragan Prokic
Keyword(s):  
Primary Biliary Cirrhosis ◽  
World Literature ◽  
Pediatric Patients ◽  
Immunoglobulin M ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Antimitochondrial Antibodies ◽  
Biochemical Tests ◽  
Autoimmune Etiology

Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic liver disease of autoimmune etiology. The initial presentation of PBC is varies from asymptomatic, abnormal liver biochemical tests to overt cirrhosis. Unlike other autoimmune liver diseases, PBC has rarely been reported in childhood. We report a case of primary biliary cirrhosis in a 12-year-old girl. In addition to characteristic histology features, strongly positive antimitochondrial antibodies, increased liver enzyme levels, increased serum quantitative immunoglobulin M levels, and cholestasis were discovered. She had been treated with ursodeoxycholic acid. In the world literature, we found only few pediatric patients of primary biliary cirrhosis. Aetiology, pathogenesis, the long-term natural history, and prognosis remain obscure. Due to increased awareness of early-onset PBC, rather than typical older ones, further pediatric cases may be discovered.

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