| Primary biliary cholangitis | |
|---|---|
| Other names | Primary biliary cirrhosis |
 | |
| Micrograph of PBC showing bile duct inflammation and injury. | |
| Specialty | Gastroenterology |
| Symptoms | Tiredness, itching, yellowish skin and eyes[1] |
| Complications | Cirrhosis, liver failure, high blood cholesterol, fat soluble vitamin deficiencies[2] |
| Usual onset | Middle-aged women[3] |
| Duration | Long term[2] |
| Types | Stage 1 to 4[3] |
| Causes | Autoimmune disease[2] |
| Risk factors | Family history, other autoimmune diseases[2] |
| Diagnostic method | Blood tests, liver biopsy[4] |
| Differential diagnosis | Autoimmune hepatitis, primary sclerosing cholangitis, non-alcoholic steatohepatitis[4] |
| Treatment | Ursodeoxycholic acid, obeticholic acid, liver transplant[5] |
| Frequency | 1 in 2,500–52,000 people[6] |
Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the small bile ducts of the liver.[2] The most common symptoms are tiredness, itching, and yellowish skin and eyes.[1] The disease may worsen over decades.[5] Complications may include cirrhosis, liver failure, high blood cholesterol, and fat soluble vitamin deficiencies.[2]
Risk factors include a family history of the disease.[2] It is also associated with a number of other autoimmune diseases including autoimmune hepatitis, celiac disease, Raynaud disease, and scleroderma.[2] The underlying mechanism involves a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up and damage the liver.[2] Diagnosis is often based on blood tests, such as anti-mitochondrial antibodies and alkaline phosphatase, or a liver biopsy.[4]
Initial treatment is generally with ursodeoxycholic acid (UDCA) which slows the progression of the disease.[5] Obeticholic acid (OCA) may also be used.[5] Other efforts revolve around the management of complications.[3] In those who develop liver failure, a liver transplant may be indicated.[5]
PBC affects around 1 in 2,500 to 1 in 52,000 people.[6] It occurs 9 times more often in women than men.[3] Those between the ages of 30 and 60 are most commonly affected.[3] It has been described since at least 1851 and was named "primary biliary cirrhosis" in 1949.[7][8] As cirrhosis is a feature only of advanced disease, a name change to "primary biliary cholangitis" was accepted in 2014.[9][10][11]
References edit
- ^ a b "Primary Biliary Cholangitis". NORD (National Organization for Rare Disorders). Retrieved 11 February 2021.
- ^ a b c d e f g h i "Definition & Facts of Primary Biliary Cholangitis (Primary Biliary Cirrhosis) | NIDDK". National Institute of Diabetes and Digestive and Kidney Diseases. Retrieved 12 February 2021.
- ^ a b c d e Pandit, S; Samant, H (January 2020). "Primary Biliary Cholangitis". PMID 29083627.
{{cite journal}}: Cite journal requires|journal=(help) - ^ a b c "Primary Biliary Cholangitis". NORD (National Organization for Rare Disorders). Retrieved 12 February 2021.
- ^ a b c d e "Primary biliary cholangitis". rarediseases.info.nih.gov. Retrieved 12 February 2021.
- ^ a b Boonstra K, Beuers U, Ponsioen CY (2012). "Epidemiology of Primary Sclerosing Cholangitis and Primary Biliary Cirrhosis: A Systematic Review". Journal of Hepatology. 56 (5): 1181–1188. doi:10.1016/j.jhep.2011.10.025. PMID 22245904.
- ^ Dauphinee, James A.; Sinclair, Jonathan C. (July 1949). "Primary Biliary Cirrhosis". Canadian Medical Association Journal. 61 (1): 1–6. PMC 1591584. PMID 18153470.
- ^ Saeian, Kia; Shaker, Reza (2016). Liver Disorders: A Point of Care Clinical Guide. Springer. p. 253. ISBN 978-3-319-30103-7.
- ^ PBC Foundation (UK). "PBC Name Change". Retrieved 27 Jan 2017.
- ^ PBCers Organization. "Primary Biliary Cirrhosis Name Change Initiative" (PDF).
- ^ Tazuma, Susumu; Takikawa, Hajime (2016). Bile Acids in Gastroenterology: Basic and Clinical. Springer. p. 110. ISBN 978-4-431-56062-3.