Primary biliary cirrhosis, also known as primary biliary cholangitis occurs when your liver’s bile ducts get destroyed slowly. That is the main primary biliary cirrhosis cause. Bile is the fluid produced in the liver that aids digestion and elimination of cholesterol, old red blood cells, and toxins. When the ducts for this bile get damaged, they accumulate in the liver and may sometimes result in an irreversible scar medically termed cirrhosis.
This disease is considered to be an autoimmune disease meaning your immune system is making a costly mistake of attacking healthy tissue and cells. Researchers are suggesting that a combination of environmental and genetic factors triggers this health condition.
Primary biliary cirrhosis develops slowly. Some medications can slow down its symptom progression especially if you start early treatment. Most times when this disease is diagnosed there are no noticeable symptoms. The diagnosis may even be made through blood tests conducted for other reasons. It takes five to twenty years for symptoms to start. Those who at their diagnosis already experience symptoms will have a poorer outcome because the disease has been untreated at its early stages and is now more developed.
Symptoms
The common early signs and symptoms of this health condition are fatigue, dry mouth, and eyes, and itchy skin.
Symptoms that may develop at later periods include:
- Upper right abdomen pain
- Spleen swelling
- Joint, muscle, or bone (musculoskeletal) pain
- Swollen ankles and feet (edema)
- Ascites
- Fatty deposits around the skin of the eyelids, eyes, creases of your palm, knees, elbows, or soles.
- Yellowing of the eyes and skin
- Skin darkening unrelated to sun rays exposure
- Brittle and weak bones which may cause fractures
- High cholesterol levels
- Diarrhea, greasy stools may be noticed
- Underactive thyroid
- Weight loss
Primary Biliary Cirrhosis Cause
As there are no clear causes of primary biliary, many experts concluded that it should be classified as an autoimmune disease.
Inflammation of the liver starts when specific white blood cell types known as T cells or T lymphocytes begin to accumulate in your liver. Normally, they are immune cells that detect and protect your body from germs attack, but in this disease, the cells, instead of fighting for your protection, begin to attack your very own healthy cells, the ones that line up the liver ducts.
This inflammation spreads out even if it starts with the smallest bile ducts, it will eventually reach out to other liver cells damaging them. As these cells die, scar tissues are formed and can cause cirrhosis. Cirrhosis is described as liver tissue scarring, which prevents the liver from functioning well.
Risk Factors
Some factors may increase the risk of you developing primary biliary cholangitis:
- Sex: Women are mostly affected by this health condition
- Age: People within the ages if their to sixty are more prone to develop theses symptoms
- Genetic factors: If there is a family history of the diseases, your chances of getting it is increased.
- Geography: Primary biliary cirrhosis mostly happens in North America and Northern Europe.
Researchers also think that specific environmental factors combined with genetic factors can trigger the disease. Environmental factors include infections by:
- Fungi, parasites or bacteria
- Smoking
- Toxic chemicals
Prevention
Several complications can develop from primary biliary cirrhosis, such as:
- Portal hypertension
- Splenomegaly
- Gallstones
- Enlarged veins
- Liver cancer
- Vitamin deficiencies
- Weak bones
- Hepatic encephalopathy
- Increased disease risk
You can work together with your doctor to prevent these complications, but how?
Portal hypertension increases portal vein pressure. Your doctor most likely will monitor and screen you for this increased pressure and also for enlarged veins.
Weak bones can be improved or prevented by engaging in activities that can help in increasing the density of your bone. Vitamin D and calcium supplements can also be administered.
Vitamin deficiencies may be addressed by using recommended Vitamin D, E, A, K supplements to enhance your vitamin levels. You should avoid taking any nutritional or herbal supplement, which your doctor does not recommend for you.
Treatment
Currently, this disease has no cure, but some medications can slow down its progression and avoid further complications.
- Ursodeoxycholic acid: This medicine is also known as ursodiol, and it is usually the first point of call in treating primary biliary cirrhosis. What it does is that it assists the passage of bile in your liver. Ursodeoxycholic acid is not a cure, but it appears to reduce the scarring of the liver and improve its function. It may not help relieve fatigue and itching, and side effects like diarrhea, hair loss, and weight gain may be experienced.
- Obeticholic acid: This newest medication for primary biliary cirrhosis is FDA approved. The results of studies suggest that it can improve the function of the liver when taken alone or with ursodiol drug for twelve months.
- Fibrates: Researchers are not sure about the exact mechanism of action for this drug, but when given with ursodiol it helps with the reduction of itching and liver inflammation in people. There is a need for more studies on this drug to ascertain its long term effects.
- Other medications: There are other drugs under study or used in the treatment of this condition with mixed outputs. These drugs include colchicine (colcrys) and methotrexate (recall).
- Liver transplant: If the disease has gone beyond the control medications, then the next option is to consider having a liver transplant to prolong the patient’s life. What happens during a liver transplant is that the diseased liver is replaced with a healthy liver. There are long term results associated with this procedure for primary biliary cirrhosis patients. However, there are cases where the disease resurfaces after several years.
Word of Advice
Some Primary biliary cirrhosis causes can be avoided by leading a healthy lifestyle right from your younger days. Limit your alcohol intake, eat healthily, exercise often, and don’t take random medications without first consulting your doctor. Live your life within the parameters of healthy restrictions. Do it for the long term.
