Primary Biliary Cholangitis (PBC), formerly known as primary biliary cirrhosis, is a rare chronic autoimmune disease that leads to the gradual destruction of the small bile ducts in the liver. Complications include end-stage liver disease, metabolic bone disease and nutritional deficiencies. It tends to affect more women than men.
Symptoms
In the early stages of PBC, individuals may not experience any symptoms. However, as the disease progresses, symptoms may include but not limited to:
Fatigue
Itching
Dry eyes and mouth
Swelling of the spleen
Yellow skin and eyes (jaundice)
Diarrhea, fat in stools
Fluid in the abdomen
Edema (Swelling in the feet and ankles)
High cholesterol
Weight loss
Cause
The exact cause of PBC is not clearly understood.
Diagnosis
Diagnosis and assessment of PBC involves a combination of medical history, physical examination, and laboratory tests. Additional investigations such as imaging,, bone densitometry, liver tissue elastography and liver biopsy may be required.
Treatment
Treatment goals include reducing disease progression as well as the management of symptoms and complications. Oral medications including ursodeoxycholic acid and obeticholic acid may help slow the progression of PBC. Oral medications such as cholestyramine may help reduce itch. Most patients will benefit from a balanced diet as well as multivitamins, calcium and vitamin D supplementation. For patients with cirrhosis, liver cancer and variceal screening is recommended. In severe cases, liver transplantation may be considered.