Serum lysozyme activities and semiquantitative analysis of tissue lysozyme distribution were studied in patients with primary biliary cirrhosis (PBC), chronic hepatitis (CH), miscellaneous liver diseases, and normal subjects. Serum lysozyme was significantly raised in PBC and CH. Portal venous blood has similar lysozyme activities to peripheral venous blood in a group of various liver diseases....

BACKGROUND Primary biliary cirrhosis (PBC) is a disease with genetic and environmental pathogenetic background. Chemicals, infectious agents, hormone therapy, reproductive history and surgical interventions have been implicated in the induction of PBC. Familial PBC has been documented in first degree relatives (FDR). Most cohort studies are genetically heterogeneous. Our study aimed to determin...

Approximately 5% to 10% of patients with features otherwise consistent with primary biliary cirrhosis (PBC) lack antimitochondrial antibodies (AMA). Most of these patients have other autoantibodies, a syndrome recently named "autoimmune cholangitis." We report our experience in patients with AMA-negative PBC treated with ursodeoxycholic acid (UDCA) and/or liver transplantation (OLT). The study ...

Background—Primary biliary cirrhosis (PBC) is an autoimmune disease in which the pathogenesis of progressive liver injury is poorly understood. Aim—To provide novel insights into the pathogenesis of PBC related liver injury using cDNA array analysis, which simultaneously examines expression of many genes. Methods—Utilising cDNA arrays of 874 genes, PBC was compared with primary sclerosing chola...

The true prevalence of PBC in RA is not well known. Herein, we report an unusual case of a patient with PBC and RA, and discuss the association between these two diseases. PBC should be ruled out in the differential diagnosis of patients with RA having abnormal liver function tests.

Primary biliary cirrhosis (PBC) is an autoimmune chronic cholestatic liver disease, histopathologically charac­ terized by portal inflammation and immune­mediated destruction of the intrahepatic bile ducts within the portal tracts and epithelioid granulomas around damaged bile ducts. The loss of bile ducts leads to decreased bile secretion and the retention of toxic substances within the liver,...

Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease that mainly targets the cholangiocytes of the interlobular bile ducts in the liver. The condition primarily affects middle-aged women. Without treatment, PBC generally progresses to cirrhosis and eventually liver failure over a period of 10-20 years. PBC is a rare disease with prevalence of less than 1/2000. PBC is tho...

Recent genome-wide association studies suggest that genetic factors contribute to primary biliary cirrhosis (PBC) susceptibility. Although several reports have demonstrated that the interleukin (IL) 12 signaling pathway is involved in PBC pathogenesis, its precise genetic factors have not been fully clarified. Here, we performed an association analysis between IL12A, IL12RB, and signal transduc...

The automimmune liver disease primary biliary cirrhosis (PBC) is characterised by serum autoantibodies directed at mitochondrial and nuclear antigens (seen in most patients and a subset of patients, respectively). The antimitochondrial antibodies (AMA) characteristic of PBC are directed at members of the 2-oxoacid dehydrogenase components of multienzyme complexes; in particular, the E2 and E3 b...

Primary biliary cirrhosis (PBC) and systemic sarcoidosis are granulomatous diseases of unknown etiology whose hepatic manifestations may infrequently be imitative of one another. Described herein is the first reported case in the medical literature of systemic sarcoidosis developing after liver transplantation for PBC. The presented patient, who suffered from typical clinical, laboratory, and p...