Primary Biliary Cirrhosis (PBC) is a potentially serious medical condition in which there is gradual destruction of bile ducts in the liver resulting in numerous complications. Lack of bile secretion in intestine and retention of bile secretion within blocked bile duct causes several complications. In this article, we will discuss in detail about some of the causes, symptoms, and treatment of Primary Biliary Cirrhosis (PBC).
How Is Primary Biliary Cirrhosis (PBC) Defined?
- Bile is secreted by the liver and carried to Gall Bladder through bile duct.
- Bile is stored in gall bladder and periodically discharged in to duodenum.
- Bile in intestine is responsible to aggregate fat molecules to create larger surface area, which assists in digestion by pancreatic enzymes.
- Bile within liver and gall bladder also mediate excretion of bilirubin and metabolic discharge product of red blood cells.
- Autoimmune disease in few cases causes inflammation of bile duct and following inflammation bile duct heals with fibrous tissue or scar tissue.
- Scarring of bile duct causes obstruction of bile, which results in cirrhosis.
- Lack of bile in duodenum and obstruction to forward flow of bile causes abnormal fat absorption and accumulation of physiological toxic substances like bilirubin and molecule of hemoglobin.
- The active immune system in autoimmune disease activates lymphocytes to destroy normal own body cells like endothelial cell of bile duct
- The destruction of bile duct is slow and early initiation of treatment results in slow progress and better prognosis.
Causes of Primary Biliary Cirrhosis (PBC)
Primary Biliary Cirrhosis (PBC) Due to Autoimmune Disease
- Antimitochondrial Antibodies 1-
- Acts against pyruvate dehydogenese enzyme in mitochondria.
- Enhance T Cell (Lymphocytes: White Blood Cells)Activities-
- Primary Biliary Cirrhosis is said to begin when T cells start to accumulate in the liver.
- T cells are WBCs that form a part of the immune system response.
- In usual circumstances, T cells are able to recognize and defend against bacteria and fungus but in the case of Primary Biliary Cirrhosis these cells start invading and destroying the cells, which line the bile ducts in the liver.
Primary Biliary Cirrhosis (PBC) Due to Genetic Abnormality
- Associated with gluten sensitive enteropathy
- Seen in family members mostly first-degree relatives. 2
- Gene encoding- the protein of the gram-negative alphabacterium reacts with mitochondrial protein resulting in inflammation and scarring of bile duct.
- Primary biliary cirrhosis was found to be associated with immunomodulatory gene.3
Risk Factors for Primary Biliary Cirrhosis (PBC)
Some of the Risk Factors for Primary Biliary Cirrhosis (PBC) are:
- Sex: Primary Biliary Cirrhosis affects females more than males.
- Age: Primary Biliary Cirrhosis mostly occurs in individuals between 30 and 60 years of age.
- Infection: Researchers are of the belief that Primary Biliary Cirrhosis can be caused by bacterial or parasitic infection
- Chronic smoker 2
- Urinary tract infection 2, 4
Symptoms Of Primary Biliary Cirrhosis (PBC)
Some of the symptoms of Primary Biliary Cirrhosis are as follows:
Non-Specific Symptoms of Primary Biliary Cirrhosis (PBC)
- Dry mouth
- Dry skin
- Dry eyes
- Loss of appetite
Specific Symptoms of Primary Biliary Cirrhosis (PBC)
- Increased level of bilirubin in blood-
- Itching of the skin
- Dry Eye
- Dry mouth
- Portal hypertension
- Splenomegaly (enlarge spleen)
- Esophageal varieces ( engorge vein in lower end of esophagus)
- Cholesterol deposits in skin-
- Xanthoma over arms and upper body
- Xanthoma around eyes
- Enlarged liver
- Abdominal pain
- Retention of fluid-
- Edema- feet and ankle
- Hepatic encephalopathy
- Somnolence and unconscious
- Liver failure
Diagnosis of Primary Biliary Cirrhosis (PBC)
Some Of the Procedures to Diagnose Primary Biliary Cirrhosis (PBC) are:
- Liver function tests: This test will identify the functioning of the liver
- Blood draws to look for autoimmune disease: In case of Primary Biliary Cirrhosis, a routine blood draw will reveal anti-mitochondrial antibodies.
- Imaging Studies like CT/MRI can be done to look at the liver for signs of inflammation and scarring
- Liver Biopsy: This can be done to look at the extent of the damage caused to the liver due to Primary Biliary Cirrhosis.
Treatment for Primary Biliary Cirrhosis (PBC)
- There is no definitive treatment for Primary Biliary Cirrhosis, the main aim of treatment is to slow down the disease progression and controlling the symptoms and prevent complications.
Therapeutic Medications for Primary Biliary Cirrhosis (PBC)
- Improve Bile Circulation:
- Ursodiol (Usodeoxycholic acid)- The primary medication used to slow down and prevent cirrhosis is Ursodiol.5 This is a bile acid, which promotes movement of bile through the liver. This is primarily the first line of therapy for Primary Biliary Cirrhosis.
- Reduced Level Of Bilirubin
- Cholesteramine- improves itching by reducing level of bilirubin in serum.
- Naltrexone- increases absorption of bile in intestine.
- Rifampicin- increases absorption of bile in intestine.
- Treat Fatigue-
- Provigil (modafinil)- improves symptoms of fatigue.
- Treatment of Esophageal Varieces-
- Surgical treatment
- Treatment Of Liver Failure-
- Liver Transplant-
- The next and the only definitive treatment as of now is liver transplant.
Complications of Primary Biliary Cirrhosis (PBC)
Complications Of Primary Biliary Cirrhosis Are:
- Portal hypertension
- Esophageal Varieces
- Liver cancer
- Vitamin deficiencies
- Memory problems
Home Remedies for Primary Biliary Cirrhosis (PBC)
Home Remedies for Symptomatic Relief-
- Consumption of foods less in sodium
- Adequate exercises
- Avoid or even abstain from alcohol
1. Pathophysiology and recent findings of primary biliary cirrhosis complicated by systemic sclerosis.
Ohira H1, Watanabe H.
Hepatol Res. 2013 Dec 6.
2. Questionnaire based assessment of risk factors for primary biliary cirrhosis.
Lammert C1, Nguyen DL, Juran BD, Schlicht E, Larson JJ, Atkinson EJ, Lazaridis KN.
Dig Liver Dis. 2013 Jul;45(7):589-94.
3. Association of primary biliary cirrhosis with variants in the CLEC16A, SOCS1, SPIB and SIAE immunomodulatory genes.
Hirschfield GM1, Xie G, Lu E, Sun Y, Juran BD, Chellappa V, Coltescu C, Mason AL, Milkiewicz P, Myers RP, Odin JA, Luketic VA, Bacon B, Bodenheimer H, Liakina V, Vincent C, Levy C, Pillai S, Lazaridis KN,
Amos CI, Siminovitch KA.
Genes Immun. 2012 Jun;13(4):328-35. doi: 10.1038/gene.2011.89. Epub 2012 Jan 19.
4. An increased risk of urinary tract infection precedes development of primary biliary cirrhosis.
Varyani FK1, West J, Card TR.
BMC Gastroenterol. 2011 Aug 26;11:95. doi: 10.1186/1471-230X-11-95.
5. Fibrate treatment for primary biliary cirrhosis.
Cuperus FJ1, Halilbasic E, Trauner M.
Curr Opin Gastroenterol. 2014 May;30(3):279-86.