Primary biliary cirrhosis
| Primary biliary cholangitis | |
|---|---|
| Synonyms | Primary biliary cirrhosis |
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| Micrograph of PBC showing bile duct inflammation and injury. H&E stain. | |
| Classification and external resources | |
| Specialty | Gastroenterology |
| ICD-10 | K74.3 |
| ICD-9-CM | 571.6 |
| OMIM | 109720 |
| DiseasesDB | 10615 |
| MedlinePlus | 000282 |
| eMedicine | med/223 |
| Patient UK | Primary biliary cholangitis |
| MeSH | D008105 |
Primary biliary cholangitis (PBC), also known as primary biliary cirrhosis, is an autoimmune disease of the liver. It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis. Further slow damage to the liver tissue can lead to scarring, fibrosis, and eventually cirrhosis.
Common symptoms are tiredness, itching and, in more advanced cases, jaundice. In early cases, there may only be changes in blood tests.
PBC is a relatively rare disease, affecting up to 1 in 3–4,000 people. It is much more common in women, with a sex ratio of at least 9:1 female to male.
The condition has been recognised since at least 1851 and was named "primary biliary cirrhosis" in 1949. Because cirrhosis is a feature only of advanced disease, a change of its name to "primary biliary cholangitis" was proposed by patient advocacy groups in 2014.
People with PBC experience fatigue (80%) that leads to sleepiness during the daytime; more than half of those have severe fatigue. Itching (pruritus) occurs in 20–70%. People with more severe PBC may have jaundice (yellowing of the eyes and skin). PBC impairs bone density and there is an increased risk of fracture.Xanthelasma (skin lesions around the eyes) or other xanthoma may be present as a result of increased cholesterol levels.
PBC can eventually progress to cirrhosis of the liver. This in turn may lead to a number of symptoms or complications:
People with PBC may also sometimes have the findings of an associated extrahepatic autoimmune disorder such as rheumatoid arthritis or Sjögren's syndrome (in up to 80% of cases).
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Wikipedia