Liver biopsy in primary biliary cirrhosis: Clinicopathological data and stage

Abstract

The purpose of the present study was to characterize histopathological lesions in primary biliary cirrhosis (PBC) and to assess the relationship between histopathological lesions and biochemistry. Liver biopsies of 252 patients with PBC were investigated. A laboratory database was established. Histopathological characterization was performed. Relationships between detailed histopathological features and biochemistry were calculated statistically. Combining the data, a PBC group, consisting of an anti-mitochondrial antibody (AMA)-positive and -negative subgroup, and an overlap group were defined, with a female preponderance of >90% and higher activity of aspartate aminotransferase (AST) in the overlap group. Histopathological changes were characteristic in >80%. Periductal concentric fibrosis, lobular granuloma formation and steatosis were frequently remarkable. Correlations were found between alanine aminotransferase activity and modified hepatitis activity index in the overlap group and the AMA-positive group. A positive significant relationship was demonstrated between mean AST activity and portal fibrosis for the AMA-positive group. A highly significant positive link was seen between mean concentration of bilirubin and stage of fibrosis. Biochemistry reflects only in part the degree of severity of histopathological lesions in PBC. Histopathology indicates comorbidity in a high percentage of patients.

Risk Factors and Prediction of Long-term Outcome in Primary Biliary Cirrhosis

“…Histologically, in PBC there is a characteristic chronic inflammatory infiltrate that surrounds and invades biliary ductules in the portal areas of the liver, called chronic nonsuppurative destructive cholangitis, together with granuloma formation (12). PBC is classified into four histological stages: stage one, inflammation and/or abnormal connective tissue confined to the portal areas; stage two, inflammation and/or fibrosis confined to portal and periportal areas; stage three, bridging fibrosis and stage four; cirrhosis (12).…”

“…PBC is classified into four histological stages: stage one, inflammation and/or abnormal connective tissue confined to the portal areas; stage two, inflammation and/or fibrosis confined to portal and periportal areas; stage three, bridging fibrosis and stage four; cirrhosis (12).…”

Presence of disease specific autoantibodies against liver sinusoidal cells in primary biliary cirrhosis

“…Additionally, in other studies, in addition to the intense staining of the apical surface of BECs, positive staining was observed in a subset of macrophages in portal lymph nodes [6] and in hepatocytes [7] . In addition to AMAs, which are the hallmark of PBC, antinuclear antibodies (ANAs) have also been detected in 30% of PBC patients [8][9][10] . Two PBC-specific ANA immunofluorescence patterns have been identified [11,12] : "multiple nuclear dots", corresponding to the antigens Sp100 and Sp140, promyelocytic leukemia (PML) nuclear body proteins and small ubiquitin-like modifiers (SUMOs) [13,14] , and "nuclear membrane" (rim), caused by anti-nuclear envelope antibodies (ANEAs), such as gp210 and nucleoporin p62 [15,16] .…”

Adult idiopathic cholestasis: a condition more common in the Canadian Inuit?

“…In the present study, AMA negative PBC was excluded if florid duct lesions were absent on liver biopsy [1]. For those patients who had not undergone liver biopsy, the diagnosis was deemed unlikely by the absence of the classic triad for AMA negative PBC: high serum cholesterol, ceruloplasmin and IgM levels [2].…”