Primary biliary cholangitis (PBC), previously referred to as primary biliary cirrhosis, is an autoimmune and chronic liver disorder in which bilary ducts are damaged. PBC is more common in women than in men, and usually arises at 40-50 years of age. Symptoms of PBC may include chronic itchy skin, fatigue, dry eyes and mouth, abdominal pain, jaundice, and arthralgia. The combination of the following factors could increase incidence of PBC:
genetics, sex, age, environment factors such as smoking, infections, toxic materials.
Panel Test:
● Hepatic Function tests:
o Albumin
o ALP, AST, ALT,
o bilirubin direct and total
o protein
● CBC and Automated Differential
● Prothrombin Time/INR
● Autoantibodies:
✔ Autoimmune Liver Disease:
o AMA, IgG
o liver-kidney microsome-1 antibody, IgG
o F-actin (smooth muscle) antibody, IgG
o SMA, IgG
o Mitochondrial M2 antibody, IgG
o Antinuclear antibody (ANA) with HEp-2
o Centromere antibody, IgG
● Immunoglobulin Tests
o Immunoglobulin M and G (IgG, IgM)
● Other Serologic Tests:
o HBV antigen
o HCV antibody
o Hyaluronic Acid, Serum
o Cholesterol
o Thyroid Stimulating Hormone
o Vitamin A (Retinol)
o Vitamin D, 25-Hydroxy
o Vitamin E, Serum or Plasma
o Prothrombin Time/International Normalized Ratio
References
1. Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019; 69(1): 394-419. PubMed
2. Hirschfield GM, Dyson JK, Alexander GJ, Chapman MH, Collier J, Hübscher S, Patanwala I, Pereira SP, Thain C, Thorburn D, Tiniakos D, Walmsley M, Webster G, Jones DE. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut. 2018; 67(9): 1568-1594. PubMed
3. European Association for the Study of the Liver (EASL). EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017; 67(1): 145-172. PubMed
4. Bowlus CL, Gershwin E. The diagnosis of primary biliary cirrhosis. Autoimmun Rev. 2014; 13(4-5): 441-4. PubMed
5. Liberal R, Grant CR, Sakkas L, Bizzaro N, Bogdanos DP. Diagnostic and clinical significance of anti-centromere antibodies in primary biliary cirrhosis. Clin Res Hepatol Gastroenterol. 2013; 37(6): 572-85. PubMed
6. Kwo PY, Cohen SM, Lim JK. ACG Clinical Guideline: Evaluation of Abnormal Liver Chemistries. Am J Gastroenterol. 2017; 112(1): 18-35. PubMed
