Biliary cirrhosis is an autoimmune disease that affects the liver. Bile, also known gall is a greenish fluid produced by the liver that aids in digestion. Bile released by the liver is carried to the small intestine for food digestion through bile ducts. In biliary cirrhosis the small bile ducts undergo progressive destruction. This progressive destruction is due to an autoimmune response. Due to destruction of bile ducts the bile and other toxins accumulate in the liver. This build-up of toxins and bile in the liver causes the scarring or fibrosis of the liver tissue eventually. Symptoms of biliary cirrhosis are not noticeable in the early stages of the disorder. It may take about 5 to as long as 20 years for the symptoms to be apparently visible. If diagnosed for the disease in its early stages, there may not be any evident symptom but may also get diagnosed when tests for other disease is conducted. Some common symptoms that occur in the early stages are itchy skin, fatigue and dry mouth and eyes. Signs and symptoms that develop in the later stages are swelling of the spleen, pain in upper right abdomen, edema, musculoskeletal pain, build-up of bile in the abdomen causing ascites, fatty deposits on the eyelids, soles, palms, knees or elbows, jaundice, high cholesterol, weight loss, hypothyroidism, osteoporosis and hyperpigmentation. The cause of the disease is still not very clear. Researchers believe that the T lymphocytes of white blood cells start accumulating in the liver. The T lymphocytes help in fighting against any harmful agent called antigen that gets into the body. In biliary cirrhosis, these cells mistakenly attack the healthy cells of the bile ducts and destroy them. Although there is no permanent cure for the disease but treatment options are available to reduce and manage the symptoms and slow down the progression of bile duct destruction.
Treatment options for biliary cirrhosis includes medications like ursodeoxycholic acid (UDCA) which is also popular by the name of ursodiol (Urso, Actigall) that maibnly functions to move the bile through the liver, improving liver function and reducing scarring of the liver tissues. The most recent medication used for treatment of the disease is obeticholic acid (Ocaliva) which when used in combination with ursodiol for about a year has been shown to improve liver function. Fibrates (Tricor) has been found to reduce itching and inflammation of liver when taken with UDCA. There are other medications like colchicine (Colcrys) and methotrexate (Trexall) that can be used for treating the disease. In case there is no result visible with drugs and medication, liver transplant is used to slow the process of liver damage thereby helping in extending the life of the patient. Symptoms can be managed using certain medication that reduces fatigue like modafinil (Provigil). Itching can be treated using antihistamines like diphenhydramine and loratidine. Other medications that are used against itching are cholestyramine (Questran), rifampin (Rifadin) which is an antibiotic that stops itching; opioid antagonists containing naloxone (Evzio, Bunavail) can also help in relieving itching. Dry mouth and eyes can be treated using saliva substitutes and artificial tears.
Person showing the symptoms of the disease like excessive itching, excessive fatigue and long spells of dry mouth and dry eyes may require you to get the diagnostic tests done. Tests include blood tests that examine cholesterol in blood, enzyme levels in the liver, antibodies. If the person is diagnosed positive, he/she will be eligible for the treatment.
People if not diagnosed with the disease are not eligible for the treatment.
Yes, there are side effects like UDCA can cause weight gain, diarrhoea and hair loss and medication against itching like cholestyramine may work well for people but have a very unpleasant taste.
Post treatment guidelines for biliary cirrhosis are avoiding alcohol completely, exercise regularly and avoiding sea-foods that may cause bacterial infection in the liver.
The disease can never be cured. Easing the symptoms can be spontaneous and a liver transplant can prolong the life of the patient for at least five more years.
The price of biliary cirrhosis treatment in India ranges from Rs. 1 lakh to Rs. 20 lakh.
No, the results of the treatment are not permanent.
There are not much alternative for the treatment. Still some lifestyle changes and home remedies that can be followed are avoiding alcohol completely, seafoods and exercising regularly may slow down the destruction process of the bile ducts and the liver.