Primary biliary cholangitis (PBC)

Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, was renamed because most people are diagnosed before cirrhosis develops. The disease causes the immune system to gradually damage the small bile ducts in the liver, leading to a buildup of bile that can eventually result in liver damage and cirrhosis if left untreated.

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. 

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

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.

• Risk factors for recurrent primary biliary cirrhosis after liver transplantation: A systematic review and meta-analysis (2021)
• Empagliflozin eliminates refractory ascites and hepatic hydrothorax in a patient with primary biliary cirrhosis (2021)
• Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis (2021)
• Association of bezafibrate with transplant-free survival in patients with primary biliary cholangitis (2021)