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.

scholarly journals A Case of Rheumatoid Arthritis and Limited Systemic Sclerosis Overlap Successfully Treated with Tocilizumab for Arthritis and Concomitant Generalized Lymphadenopathy and Primary Biliary Cirrhosis

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Eiko Saito ◽  
Shinji Sato ◽  
Shinichi Nogi ◽  
Noriko Sasaki ◽  
Naofumi Chinen ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Lymph Node ◽  
Systemic Sclerosis ◽  
Primary Biliary Cirrhosis ◽  
Interleukin 6 ◽  
Lymph Node Biopsy ◽  
Biliary Cirrhosis ◽  
Node Biopsy ◽  
Limited Systemic Sclerosis ◽  
Generalized Lymphadenopathy

A 57-year-old woman with rheumatoid arthritis (RA) and limited systemic sclerosis (lSSc) was suspected to have lymphadenopathy and primary biliary cirrhosis (PBC). Lymph node biopsy showed reactive follicular lymphadenopathy with intrafollicular plasmacyte infiltration that was interleukin-6 positive by immunohistostaining. Because of gradually worsening arthritis, tocilizumab was administered and arthritis improved markedly. Interestingly, lymphadenopathy and PBC improved simultaneously. This suggested that interleukin-6 might play an important role in reactive lymphadenopathy and PBC associated with RA/lSSc.

Metabolism of vitamin D in patients with primary biliary cirrhosis and alcoholic liver disease

1985 ◽  
Vol 69 (5) ◽  
pp. 561-570 ◽  
Author(s):  
E. Barbara Mawer ◽  
H. J. Klass ◽  
T. W. Warnes ◽  
Jacqueline L. Berry
Keyword(s):  
Vitamin D ◽  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Alcoholic Liver Disease ◽  
Vitamin D3 ◽  
Control Group ◽  
Biliary Cirrhosis ◽  
Vitamin D2 ◽  
Dihydroxyvitamin D3 ◽  
Poor Renal Function

1. The metabolism of isotopically labelled vitamin D2 and D3 has been investigated in eight patients with primary biliary cirrhosis and in five controls. The concentration of labelled vitamin D2 was lower than that of vitamin D3 in serum of patients with primary biliary cirrhosis on days 1 and 2 after intravenous injection (P < 0.005 and P < 0.05, respectively) but no difference was seen in controls. 2. Similar amounts of labelled 25-hydroxyvitamin D2 and D3 were seen in serum of the control group; the same pattern was observed in the primary biliary cirrhosis group, and no significant differences were observed between the two groups. 3. In both control and primary biliary cirrhosis groups, the serum concentration of labelled 24,25-dihydroxyvitamin D2 exceeded that of 24,25-dihydroxyvitamin D3 (significant for controls on day 2, P < 0.02) but concentrations in the two groups were not different. 4. Concentrations of labelled 25,26-dihydroxyvitamin D3 were significantly higher than those of 25,26-dihydroxyvitamin D2 in the primary biliary cirrhosis group at all times and in the control group on days 2 and 3. Both 25,26-dihydroxyvitamin D2 and D3 were higher in the serum of patients with primary biliary cirrhosis than in controls (significant on day 1, P < 0.05). 5. Urinary excretion over days 0–3 of radioactivity from both vitamins D2 and D3 was significantly higher in the primary biliary cirrhosis group than in controls: 12.03 vs 1.80% for vitamin D2 and 8.98 vs 1.76% for vitamin D3(P < 0.005). Vitamin D2-derived urinary radioactivity in primary biliary cirrhosis correlated strongly with serum bilirubin (P = 0.005). 6. The metabolism of labelled vitamin D3 was studied in seven patients with alcoholic liver disease, three of whom showed low serum concentrations of labelled 25-hydroxyvitamin D3 suggesting impaired hepatic synthesis. The 25-hydroxylation response was quantified as the relative index of 25-hydroxylation and was significantly related to two other indices of liver function. It is concluded that impaired 25-hydroxylation of vitamin D may occur in alcoholic liver disease and results from hepatocellular dysfunction. 7. Less than the predicted amounts of 1,25-dihydroxyvitamin D3 were produced in four of the seven patients with alcoholic liver disease; this defect may be attributable in part to decreased precursor 25-hydroxyvitamin D and to poor renal function.

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

Primary Biliary Cirrhosis

2015 ◽  
Author(s):  
Daniel S. Pratt ◽  
Lindsay Y. King
Keyword(s):  
Differential Diagnosis ◽  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Liver Diseases ◽  
American Association ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Common Cause ◽  
History Of

Primary biliary cirrhosis (PBC) is a progressive autoimmune disease of the liver. It is the most common cause of chronic intrahepatic cholestatic liver disease in adults. This review addresses the epidemiology, etiology and genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of PBC. Figures show the pathogenesis and natural history of PBC and histologic features of the four stages of PBC. Tables list diagnostic criteria for PBC via the American Association for the Study of Liver Diseases, differential diagnosis for PBC, medications used to treat PBC, secondary therapy for PBC, and follow-up of patients with PBC. This review contains 2 highly rendered figures, 5 tables, and 45 references.

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

A scoring system for primary biliary cirrhosis and its application for variant forms of autoimmune liver disease

2003 ◽  
Vol 38 (1) ◽  
pp. 52-59 ◽  
Author(s):  
Kazuhide Yamamoto ◽  
Ryo Terada ◽  
Ryoichi Okamoto ◽  
Youichi Hiasa ◽  
Masanori Abe ◽  
...  
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Scoring System ◽  
Autoimmune Liver Disease ◽  
Biliary Cirrhosis

scholarly journals Primary biliary cirrhosis and myopathy: an uncommon association

1999 ◽  
Vol 54 (5) ◽  
pp. 165-168 ◽  
Author(s):  
Bruno Cupertino Migueletto ◽  
Abrahão Elias Hallack Neto ◽  
Elaine Zamora Domingues ◽  
Pedro Paulo Neves de Castro ◽  
Hartmut Stocker ◽  
...  
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Bile Ducts ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Muscular Weakness ◽  
Muscular Diseases ◽  
Histological Features ◽  
Intrahepatic Bile Ducts ◽  
Organ Systems

Primary biliary cirrhosis (PBC) is a cholestatic liver disease, which is characterized by a chronic inflammatory destruction of intrahepatic bile ducts. It is a rare disorder whose precise etiology is still to be elucidated. Even though the liver is the principal target of PBC, other organ systems also might be affected. Muscular involvement has rarely been described in this disease, and in the majority of cases, muscular weakness has been interpreted as polymyositis. We report the case of a 48-year-old woman suffering from classic PBC, in association with a myopathy whose histological features are distinct from the cases reported before. We also performed a MEDLINE research for PBC and concomitant muscular diseases.

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