scd163 in pbc patients - assessment of disease severity and prognosis

Conditions: Primary Biliary Cirrhosis; Liver Inflammation

Summary: primary biliary cholangitis (pbc) is an autoimmune chronic liver disease, characterised by destruction of the small intrahepatic bile ducts. scd163 is a macrophage activation marker shedded into plasma by macrophages in the liver. smr is a soluble mannose receptor. the investigators want to investigate whether scd163 and smr correlate with disease severity in patients with pbc, and whether scd163 and smr can predict short term disease progression, changes in quality of life and death in these patients.

primary biliary cholangitis (pbc, previously called 'primary biliary cirrhosis') is an autoimmune cholestatic liver disease characterized by destruction of intrahepatic bile ducts and progression to liver fibrosis and cirrhosis. in the pre-cirrhotic phase, fatigue and pruritus are the dominant symptoms. they reduce pbc patients' quality of life, but the extent to which they cause the patient to leave the work force and seek disability pension is unknown. the diagnosis of pbc is based on the presence of two of three major criteria; unexplained serum alkaline phosphatase (alp) \>1.5 times upper normal limit for more than 24 weeks, presence of anti-mitochondrial antibodies (ama), and compatible liver histology. multiple models have been conducted to predict prognosis in patients with pbc. the mayo risk score is the best validated and includes information on age, bilirubin, albumin, prothrombin time and peripheral oedema. other prognostic factors are pruritus and fatigue at diagnosis that predict the time to develop cirrhosis and its complications. in pbc, inflammation is attributed to an immune response to mitochondrial autoantigens followed by a serologic response of anti-mitochondrial antibodies (amas); and accompanied by inflammation of small bile ducts. the pathogenesis includes both cd4 and cd8 cells, which in the presence of biliary cells expressing the 2-oxo-dehydrogenase pathway (pdc-e2) activates macrophages via granulocyte macrophage colony-stimulating factor. the activated macrophages, together with amas, produce a proinflammatory response with subsequent liver inflammation and fibrosis. thus, macrophages seem to be involved in pbc disease severity and progression. however, macrophage activation markers have not previously been investigated in pbc patients. the investigators' research group have during the last years investigated the macrophage activation marker scd163. the group have shown increased levels in relation to liver fibrosis/cirrhosis in patients with chronic viral hepatitis (hbv and hcv), non-alcoholic fatty liver disease (nafld/nash) and alcoholic liver disease (alcoholic hepatitis and cirrhosis) and liver disease severity including risk of portal hypertension and development of complications and mortality. just recently the investigators' research group also demonstrated that the soluble mannose receptor (smr) and scd163 are associated with early and long-term prognosis of patients with cirrhosis and acute-on-chronic liver failure. aims: to investigate scd163 and smr as markers of fibrosis and cirrhosis in pbc patients. further, the investigators will investigate scd163 and smr as prognostic markers of short-term disease progression and impact on quality of life in patients followed in our liver centre. moreover, the patients' short-term risk of requiring disability pension will be investigated. this will improve the information available for the patients regarding their short-term prognosis.

Eligibility criteria:
inclusion criteria: * diagnosed with primary biliary cholangitis exclusion criteria: * patient under 18 years * expected lifetime below 6 months * planned liver transplantation within 6 months * cirrhosis from other causes (except autoimmune hepatitis) * liver cancer

Keywords: prognosis