Primary biliary cholangitis (PBC)
A long-term autoimmune liver disease in which the immune system slowly damages the small bile ducts inside the liver, causing bile to build up. It often shows first as fatigue and itching with a raised alkaline phosphatase, and is strongly linked to a specific autoantibody (anti-mitochondrial antibody).
What it is
Primary biliary cholangitis (formerly called primary biliary cirrhosis) is a chronic autoimmune disease in which the immune system attacks and gradually destroys the small bile ducts within the liver. Bile — the fluid the liver makes to help digest fat and to carry away waste — cannot drain properly, so it accumulates in the liver (cholestasis) and slowly damages liver tissue. Over many years this can progress to scarring (fibrosis) and, in some, to cirrhosis and liver failure, though treatment has made this much less common. It predominantly affects women in middle age. The hallmark is a raised alkaline phosphatase (and GGT), the enzymes that signal bile-duct problems, and it is very strongly associated with a specific autoantibody in the blood — the anti-mitochondrial antibody (AMA) — which is positive in the great majority of people with the disease and is central to the diagnosis; the immunoglobulin IgM is often raised too. Many people are picked up before symptoms, from an unexplained high alkaline phosphatase. When symptoms occur, the commonest and often earliest are persistent tiredness and itching (pruritus), later sometimes jaundice, dry eyes and mouth, and features of long-term liver disease. Treatment with bile-acid medication (ursodeoxycholic acid) slows progression and improves outlook, especially when started early.
Key lab markers
- Alkaline phosphatase (ALP) — the hallmark; typically raised, reflecting bile-duct injury (cholestasis).
- GGT — usually raised too, confirming the ALP is of liver/bile-duct origin.
- Anti-mitochondrial antibody (AMA) — positive in the great majority; the key diagnostic autoantibody (mentioned here as it is central to diagnosis).
- Direct (conjugated) bilirubin — may rise as the disease advances; an important prognostic marker.
- IgM — the immunoglobulin often raised in PBC.
Symptoms
- Persistent tiredness and fatigue — often the earliest symptom
- Itching (pruritus), sometimes intense
- Dry eyes and dry mouth
- Discomfort in the upper-right abdomen
- Jaundice (yellowing) as the disease advances
- Darkening of the skin, and small fatty deposits around the eyes
- Often no symptoms at all early on — found from an abnormal blood test
Related lab panels
When to discuss with a doctor
See a doctor for an unexplained raised alkaline phosphatase, persistent unexplained tiredness, or ongoing itching without a rash, as these can be early signs of PBC and are worth investigating with liver tests and the anti-mitochondrial antibody. An early diagnosis matters because bile-acid treatment slows the disease and works best when started before significant liver damage. Someone with known PBC should attend monitoring so the liver and treatment response are tracked, and should seek prompt review for new jaundice, worsening itch, or signs of advanced liver disease. Mediora.AI can surface the cholestatic pattern — a raised alkaline phosphatase and GGT, with direct bilirubin and IgM — that should prompt testing for the anti-mitochondrial antibody, but the diagnosis and treatment belong with your doctor and liver specialist.