The disease was earlier called primary biliary cirrhosis, but its new name, primary biliary cholangitis (PBC) describes it better: an autoimmune disease of the liver which may lead to cirrhosis through slow progression.
A chronic cholestatic disease with unknown aetiology characterized by inflammation that damages small bile ducts.
At the time of diagnosis the patients often have only few symptoms. Investigations are started because of increased alkaline phosphatase (ALP) concentration.
Large increase in bilirubin concentration is a sign of active or advanced disease.
Epidemiology
Incidence is 0.2-5.8 new cases/100 000 population, prevalence 10-50/100 000 population.
90% of the patients are women.
The age of onset is (20-)40-60(-70) years.
Symptoms and signs
The typical patient is a middle-aged woman with no or only few symptoms.
Abnormal ALP concentration is incidentally detected.
Possible symptoms include itch, jaundice and hepatomegaly.
Generalized itch, initially only in the evening, is usually the presenting symptom. The itch may appear during the use of oestrogens or drugs causing cholestasis, and sometimes in association with pregnancy.
Jaundice is the presenting symptom in 10% of the patients.
Hepatomegaly is detected in 50% of the patients on presentation.
Splenomegaly, xanthelasmas, or cirrhosis of the liver is detected in some patients already on presentation.
Non-specific symptoms include fatigue, articular symptoms, and dry mouth or eyes.
Osteoporosis Osteoporosis is common in advanced disease.
Budesonide (6-9 mg/day) combined with ursodeoxycholic acid sometimes helps in problematic cases (poor treatment response to ursodeoxycholic acid, substantial inflammatory findings at histology but no cirrhotic liver damage).
Liver transplantation may be required due to complications of PBC: liver failure, recurring variceal bleeding, difficult-to-manage ascites, cholestasis-related unbearable itch, etc., usually not earlier than at the age of 60 to 65 years.
Treatment and follow-up are performed in specialized care.
The frequency of follow-up checks is determined by the severity of the liver disease.
Liver function tests etc. every 3 to 12 months
Liver ultrasonography every 1 to 3 years
Prognosis
The course of the disease is better predicted than in most other cirrhoses. Increased bilirubin concentration is a sign of poor prognosis.
After the patient has become symptomatic, life-expectancy without liver transplantation is 5-10 years.