Primary biliary cirrhosis (PBC)

 

Overview

Caused by a buildup of bile (a substance used to facilitate digestion and eliminate toxins in the liver) due to progressive destruction of the bile ducts by the immune system, this disease can eventually lead to liver damage and cirrhosis.

Common Symptoms

Fatigue, itchy skin, dry eyes and mouth, and pain in the upper right abdomen, fatty deposits (xanthomas) on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees, yellowing of the skin (jaundice), hyperpigmentation, musculoskeletal pain, swollen feet and ankles, weight loss, diarrhea, and high cholesterol. 

Coexisting Diseases and Conditions

Osteoporosis, rheumatoid arthritis, Raynaud’s phenomenon, SLE, multiple sclerosis, Sjögren’s syndrome, scleroderma, polymyositis, thyroid disease, and type 1 diabetes.

Risk Factors and Prevalence

PBC occurs more often in females than males. It is commonly diagnosed in adults between 30 to 90 years of age (source). Family history of the disease is another risk factor. In addition, bacterial infections such as urinary tract infections (UTIs) have been associated with PBC. Environmental factors, including smoking cigarettes and exposure to toxic chemicals have also been associated with PBC.

Sources

  1. Article Sources and Footnotes

    1. Primary biliary cholangitis—Symptoms and causes. (n.d.). Mayo Clinic. Retrieved June 12, 2021, from https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

    2. Primary biliary cirrhosis. (2017, November 30). Nhs.Uk. https://www.nhs.uk/conditions/primary-biliary-cirrhosis-pbc/

    3. Smyk, D., Cholongitas, E., Kriese, S., Rigopoulou, E. I., & Bogdanos, D. P. (2011). Primary Biliary Cirrhosis: Family Stories. Autoimmune Diseases, 2011, 189585. https://doi.org/10.4061/2011/189585

    4. Smyk, D. S., Rigopoulou, E. I., Muratori, L., Burroughs, A. K., & Bogdanos, D. P. (2012). Smoking as a risk factor for autoimmune liver disease: What we can learn from primary biliary cirrhosis. Annals of Hepatology, 11(1), 7–14. https://doi.org/10.1016/S1665-2681(19)31481-4

    5. Sun, Y., Haapanen, K., Li, B., Zhang, W., Van de Water, J., & Gershwin, M. E. (2015). Women and primary biliary cirrhosis. Clinical Reviews in Allergy & Immunology, 48(2–3), 285–300. https://doi.org/10.1007/s12016-014-8449-4