Primary biliary cholangitis Flashcards
what is the definition of PBC?
Primary biliary cholangitis (PBC) is a chronic disease of the small intrahepatic bile ducts that is characterised by progressive bile duct damage (and eventual loss) occurring in the context of chronic portal tract inflammation.
what is the epidemiology of PBC?
more common on females
aged 45-60
what is the aetiology of PBC?
Thought to be autoimmune, high incidence of autoantibodies directed against mitochondrial antigens
Other features that point to an autoimmune aetiology in PBC include a high personal and family incidence of other autoimmune processes, including Sjogren’s syndrome, scleroderma, and coeliac disease.
what are the risk factors for PBC?
Female sex
45-60
Family history, smoking, UTIs
what is the pathophysiology of PBC?
The classic pathophysiological process in PBC is damage to, and progressive destruction of, the biliary epithelial cells lining the small intrahepatic bile ducts. Bile ducts are damaged in the context of portal tract inflammation, further supporting the concept of an autoimmune component to the disease.
what are the key presentations of PBC?
Aged between 45 and 60
Female sex
what are the signs of PBC?
Personal and family history of autoimmune diseases
Female
45-60
History of hypercholesterolaemia
Hepatomegaly
Jaundice, ascites, splenomegaly, skin pigmentation, xanthelasma
what are the symptoms of PBC?
Itchiness Fatigue Dry eyes and mouth Sleep disturbance Postural dizziness Weight loss, memory problems
what are the first line and gold standard investigations for PBC?
Alkaline phosphatase - elevated gamma-GT - elevated Bilirubin - elevated ALT - elevated Serum albumin - decreased Antimitochondrial antibody and ANA immunofluorescence ELISA tets Abdominal ultrasound scan MRI
what other tests could be done for PBC?
Prothrombin time Serum immunoglobulin Liver biopsy Upper GI endoscopy Serum alpha-fetoprotein
what are the differential diagnoses for PBC?
Obstructive bile duct lesion
Small duct primary sclerosing cholangitis
Drug induced cholestasis
how is PBC managed?
Bile duct analogue - ursodeoxycholic acid: 12-16 mg/kg/day orally given in 3 divided doses or obeticholic acid Immunomodulatory therapy Antipruritic treatment Lifestyle modification Liver transplant
how is PBC monitored?
Once patients are established on ursodeoxycholic acid it is recommended that satisfactory response is determined using clinical response criteria that have been identified and validated.
what are the complications of PBC?
Hypercholesterolaemia
Osteoporosis
Portal hypertension secondary to cirrhosis
Heptoma
what is the prognosis of PBC?
PBC is a slowly progressive condition in the majority of patients. Given the age of presentation with the disease, many patients die of other causes before reaching end-stage liver disease. Epidemiological studies, however, suggest that mortality overall is significantly increased in PBC patients
