primary biliary cholangitis

Primary biliary cholangitis (PBC) can impact both the quality of life and the survival of patients. The study aimed to determine the survival rate and associated variables in patients with PBC.

This cohort research comprised 65 patients diagnosed with PBC who were admitted to the pathology section between January 2010 and December 2019. Survival was determined by reviewing hospital medical data and following up with the patients. The impact of demographic factors, clinical, laboratory, and histopathological aspects on patient survival time was investigated using Kaplan-Meier survival analysis and Coxregression.

The average period of follow?up was 6.25 years with a standard deviation of 3.2 years. In surviving patients, the baseline bilirubin level was 2.83, but in deceased or transplanted patients, it was 8.95 (P = 0.002). The baseline albumin level was 3.99 in surviving patients and 3.66 in deceased or transplanted patients (P = 0.024). The incidence of cirrhosis in those who survived was 1.8%, but in patients who died or underwent a transplant, it was 40%. Out of 65 cases, 3 patients (4.7%) died and 7 (10%) had liver transplants. Survival rates of patients vary based on factors such as jaundice (P = 0.002), weariness (P = 0.03), cirrhosis (P < 0.001), and vitiligo (P = 0.033). There were notable variations in the average Mayo score between the two groups of patients who had liver transplantation and survived, with scores of 7.21 and 5.61, respectively.

The study found that aspartate aminotransferase and alanine aminotransferase levels, baseline and final bilirubin, albumin, antinuclear antibody, the presence of cirrhosis, and jaundice significantly influenced patient survival with PBC.

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a liver disease. This is a chronic autoimmune disease, which can worsen over time and recur periodically. If left untreated, biliary cirrhosis may lead to liver failure. Considering that PBC is a rare disease in all age groups, especially in children, the purpose of this report was to describe a girl with hepatitis A virus (HAV) who was then diagnosed with PBC. This child is the first case reported in Iran. The patient is a 7-year-old girl with a pale complexion and eyes with a yellowish cross who was referred to the doctor. Considering that this patient had hepatitis, a blood test was requested to check the level of cholesterol and liver enzymes. The absence of cholestatic liver enzymes was observed in tests. Then, an anti-mitochondrial (AMA) test was requested for the patient, the result of which was negative. Finally, with imaging and biopsy, the diagnosis of PBC was confirmed for the patient. After the definite diagnosis of the disease, the child was treated with ursodeoxycholic acid (UDCA). The child in question is suffering from two medical and immunological diseases, HAV and PBC. Since this child was first infected with HAV, it is possible that the cause of PBC was HAV.

Primary biliary cholangitis (PBC) is a condition affecting the liver and immune system. In this study, the impact of autologous bone marrow-derived mononuclear cell (BM-MNC) transplantation on PBC patients was investigated.  Sixteen eligible PBC patients participated at the National Scientific Medical Center in Astana, Kazakhstan, between 2017 and 2022, and BM-MNCs were harvested from their anterior iliac crest. After isolating and cultivating the BM-MNCs, they were infused back into the patient’s peripheral veins. Changes in BM-MNC and peripheral blood mononuclear cell (PB-MNC) phenotypes were assessed before and after a 24-hour cultivation period and 72 hours post-transplantation. We monitored liver function parameters over 6-month intervals and conducted flow cytometry analysis to assess CD markers on BM-MNCs before and after cultivation and PB-MNCs before and after transplantation. Statistical analysis included the Friedman test for liver parameters and the Wilcoxon signed-rank test for BM-MNC and PB-MNC comparisons. Our findings revealed significant reductions in liver function tests after multiple transplantations. Flow cytometry analysis before and after a 24-hour culture and autologous BM-MNC infusion revealed the expansion of specific cell populations, with significant increases in CD3+, CD4+, CD16+, CD20+, CD25+, CD34+, CD105+, CD73+, СD117+, and CD34+populations, while CD4+25+, CD34+105+, and CD4+FOXP3+ populations decreased. Interestingly, a contradictory finding was observed with a decrease in bone marrow CD34+105+ cell lines (P=0.03) alongside an increase in peripheral CD34+105+ population (P=0.03). In summary, our study shows that BM-MNC transplantation in PBC patients leads to changes in immune cell populations and liver function. These findings suggest potential therapeutic applications of BM-MNC transplantation in managing PBC and offer insights into the dynamics of immune cells associated with this treatment approach.

There are several studies reporting the therapeutic effects of Berberis vulgaris on liver diseases. This study was done with the purpose of examining the effect of B. vulgaris oxymel (BO) in patients with refractory primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC), who did not respond to current treatment.

Patients with PSC or PBC who were receiving ursodeoxycholic acid (UDCA, 13-15 mg/kg/day) for at least six months, but their serum levels of alkaline phosphatase (ALP) were still 1.5 folds higher than the normal upper limit during the last six months, were asked to participate in this quasi-experimental study. Patients were asked to take 0.5 ml/kg/day of BOtwo times a day for three months along with UDCA. At the end of the study, serum levels of ALP, aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyl transferase (GGT), total bilirubin (TB), direct bilirubin (DB), and creatinine as well as prothrombin time (PT), international normalized ratio (INR) and quality of life  (QOL) based on PBC-40 questionnaire were assessed as outcomes.

Our results showed that BO notably attenuated the serum levels of ALP, AST, ALT, GGT, TB, and DB, as well as PT and INR and significantly improved QOL.  

For first time, we showed that additional therapy with BOhas a promising effect in the treatment of refractory PSC and PBC.

Multiple sclerosis (MS) is an autoimmune disorder of the central nervous system which damages the myelin and axon. Primary biliary cholangitis (PBC) is a slow progressive liver disease with autoimmune feature in which non-purulent destructive cholangitis and interlobular bile duct destruction occur. Involvement of each of PBC and MS is thought to be related to environmental exposure in genetically susceptible persons.

Here, we aim to report 3 women 52, 27 and 51 years old with MS and PBC.

Although MS seems to have an association with some autoimmune gastrointestinal disorders such as ulcerative colitis, the concurrence of MS and PBC has been rarely reported

Primary biliary cholangitis , previously known as primary biliary cirrhosis (PBC), is the most common autoimmune diseases of the liver (ALD). Patients with PBC may present with typical biliary pattern symptoms. Also the presence of anti mitochondrial autoantibodies (AMAs) is the laboratory hallmark of PBC, which molecular target antigens are members of 2-oxoacid dehydrogenase complex of enzymes. In recent years, autoantibodies (Aab) targeting subcellular structures described as the rods and rings (R&R) pattern in HEp-2 ANA have been presented as a unique and particular case of Aab generation. These R&R structures are composed of inosine monophosphate dehydrogenase type 2 (IMPDH2), and their formation can be induced in vitro by several small-molecule inhibitors. Aab targeting these relatively unknown structures have been almost exclusively observed in hepatitis C virus (HCV) patients who have undergone treatment with pegylated interferon-a/ribavirin (IFN/RBV) therapy. Literature showed that anti-RR Aab have not been found in treatment-naïve HCV patients or in patients from any other disease. Now We present and characterized a case patient with contemporary presence of AMAs and R&R Aab in PBC, without any laboratory evidences of HCV and/or other hepatic virus infection. For our knowledge this is the first case described in the Literature. R&R Aab in patients without any clinical/laboratory signs or symptoms of Hepatitis virus and without pharmacological therapy open the window to the alternative scenario: the association of these Aab to ALD. The AMA-negative cases are an intriguing diagnostic problem. Our work demonstrated that R&R Aab can be present in PBC case.  The interesting idea suggesting suggested that R&R Aab may be present also in AMA-negative PBC and they can considered a new diagnostic tool in this specific clinical condition. Other study and cases are needed but the presence of R&R Aab linked with AMAs and PBC may be explained by alterations in immune regulation caused by autoimmunity in a particular genetic background.