The findings suggest that PBC has an autosomal dominant mode of inheritance. Familial aggregation was seen in all four HLA Class I-defined subgroups, but did not occur in HLA Class II-defined subgroups. Results from a recent clinical trial supports the hypothesis that PBC runs in families.
The etiology of PBC remains elusive; however, this article outlines the current research on the possible involvement of various infectious agents, autoimmune disorders, and environmental factors in triggering PBC. This article emphasizes the need for further investigation into this intriguing and challenging disease entity.
Liver [transplant](https://www.withpower.com/clinical-trials/transplant)ation will cure PBC in about 40% of cases, while other treatments will not improve outcomes and may increase complications. There are no randomized controlled trials comparing these treatments.
Primary sclerosing cholangitis is the primary abnormality causing PBC. The interaction between immune system and liver tissue results in inflammation and fibrosis of the liver. The autoimmune process seems to be self-limiting, because the antibodies are present at low levels and do not seem to have any effect. If PBC continues to progress, then secondary causes such as infection, bile duct cancer, and gallstones become important. There are several types of PBC, and each has its own pattern of progression.
The prevalence of PBC is 0.4% in the US population per year, with a higher prevalence in women than men. The gold standard for diagnosis of PBC is liver biopsy.
This is the first study which summarizes the most commonly used medical treatments of PBC in Denmark. Lipid-lowering drugs were often prescribed but have not been shown to prevent progression to ESCC. Statins were rarely used. Rheumatology referrals were frequent. Although rheumatologic interventions are not routinely performed in Denmark, our data show that they are frequently utilized. A prospective multicenter observational study is urgently needed to better define the necessity for these interventions.
This population-based study shows that average age of onset of PBC is equal to 49.9 ± 8.6 yr in women and 44.1 ± 6.2 yr in men, suggesting similar incidence rates for both sexes, although there seems to be an earlier age of onset of PBC in women. The findings do not support previous reports from single referral centers, where early onset of PBC was found in young patients.
Results from a recent paper, we found SBA (sclerosing cholangitis) to be one of the most common extrahepatic manifestations of PBC. However, it is not a sign of disease activity.
Autoimmune hepatitis is rare in PBC, so it should be considered in patients with abnormal liver function tests. In addition, the presence of antinuclear antibodies may support the diagnosis of autoimmune hepatitis in PBC.
Patients with PBC had significantly lower scores on health-related quality-of-life measures than controls. The functional status was also impaired. Results from a recent paper suggest that health-related quality-of-life measures should be included in the routine assessment in this patient group.
Increased serum levels of Tregs and IL-10 were found to correlate with HE in PBC patients. This information might be useful in predicting HE and reversing its severity.
The prevalence of liver cirrhosis was significantly higher in women. Alcohol consumption was more common in men than women. Age was an independent risk factor for liver cirrhosis in all groups.