The precise cause of the disease is unknown. It tends to run in families and is thought to result from abnormal autoimmune reactions. Some people with other autoimmune diseases are also found to have PBC, such as; autoimmune hepatitis, celiac disease, thyroid diseases, Raynaud’s diseases, scleroderma, etc. People with PBC have been found to have frequent urinary tract infections, particularly with Enterobacteriaceae bacteria, exhibiting the cross-reactivity between the bacterial parts and the body’s antigens. Some environmental factors may play a role in triggering the disease, such as; smoking, infections, and chemical exposure. Etc.
PBC is found globally and affects 1 in 4000 people. The cases are found more in Northern Europe and North America. In the United States, the available studies show the prevalence of around 40.2 cases per 100,000 population. Women are more likely to have the disease, particularly between 30 and 60 years of age.
The following factors may put you at increased risk of contracting the illness.
· Female gender
· Genetics; family history
· Northern European race
· Co-existence of other autoimmune diseases
· Frequent urinary tract infections
· Smoking
· Exposure to chemicals
You may not have any symptoms in the early periods of the disease, and it may be found incidentally during the routine blood tests. The typical symptoms that are present as the disease progresses are;
· Feeling unusually tired (fatigue)
· Feeling itchy all over the skin (pruritus)
· Having abdominal pain
· Your eyes and mouth may feel dry
· You may feel pain in the joints and muscles
In the advanced stages of PBC, the symptoms relate to the cirrhosis of the liver and include;
· Easy bleeding or bruising
· Nausea
· Weight loss
· Jaundice
· Swelling in your lower legs and feet called edema
· Fluid accumulation in your abdomen (ascites)
Your doctor may ask you questions about your symptoms, check physical signs, and order the following investigations.
· Blood tests: Blood test is performed to check for a specific anti-mitochondrial antibody (AMA). Its presence almost confirms PBC. Other parameters to be checked are anemia, the number of blood cells, liver enzymes and various components (ALT,AST,Alkaline Phosphatase (ALP),Bilirubin, total proteins etc), cholesterol and triglycerides, coagulation profile, and tests for hepatitis C and B viruses, etc.
· Imaging: Ultrasound, CT, MRI, and Magnetic resonance cholangiopancreatography (MRCP) scans of the abdomen can be used to assess the degree of fibrosis. The latest imaging test to determine the elasticity of the liver has been used lately, called Magnetic resonance elastography (MRE).
· Biopsy: Your doctor may suggest taking a sample of your liver tissue and checking in the laboratory for the extent and severity of fibrosis.
Some other diseases may look alike PBC;
· Autoimmune Hepatitis
· Alcoholic liver disease
· Hepatitis C or Hepatitis B chronic hepatitis
· Non-alcoholic fatty liver disease
· Biliary Obstruction
· Drug-Induced Hepatotoxicity
· Primary Sclerosing Cholangitis
· Sarcoidosis
The goal of treatment is to halt disease progression and provide symptomatic relief.
Medication: For PBC, Ursodeoxycholic acid (UDCA) is the first-line treatment. It improves the flow of bile and slows the progression of the disease. In people intolerant to UDCA, another medicine is approved, called Obeticholic acid (OCA).
Another group of medicines, called fibrates, helps control symptoms when given along with UDCA.
To control the symptoms of itching, cholestyramine, colestipol, or colesevelam are given, which are anion-exchange resins.
Fat-soluble vitamin supplements A, D, E, and K are given as people with PBC have decreased absorption of vitamins. People may also need to be evaluated for osteoporosis and be managed accordingly.
Liver transplant: if there is no improvement in the symptoms and the liver is damaged beyond healing, a liver transplant may be suggested.
For a better outcome, early diagnosis is necessary. If the treatment with UDCA is started before cirrhosis of the liver and the levels of the enzyme show improvement, the outcome is excellent, and a person can have a normal life span. However, the presence of cirrhosis on diagnosis, and abnormal liver enzyme levels, are predictors of poor prognosis. There is also an increased risk of Hepatocellular carcinoma.
Following lifestyle changes can be adopted to reduce liver damage;
· Avoid drinking alcohol
· Stop smoking
· Consume a healthy diet rich in vitamins A, E, K, vitamin D and calcium, minerals, omega 3 fatty acids, antioxidants,
· Avoid fatty and fried food, raw shellfish, and foods high in sugars and carbs.
· Employ regular exercises to improve inactivity and combat fatigue
Our clinical experts continually monitor the health and medical content posted on CURA4U, and we update our blogs and articles when new information becomes available. Last reviewed by Dr.Saad Zia on May 4th, 2023.
Primary biliary cholangitis (PBC) is an autoimmune disease that affects the liver. The old name of this disease is primary biliary cirrhosis. Inside your liver are small bile ducts that carry and transfer a digestive substance called bile. In this disease, these bile ducts are inflamed and damaged, resulting in the buildup of bile in the liver causing liver cirrhosis. The liver loses its ability to execute its functions, resulting in the symptoms of tiredness, loss of appetite, itchiness, nausea, vomiting, and unexplained weight loss. With the progression of the disease, symptoms may include jaundice, easy bruising, swelling in the lower legs, and building of fluid in the abdomen. PBC is a slowly progressive long-term illness.