Cholangiopathy

Cholangiopathy refers to diseases that affect the bile ducts, the channels that carry bile from the liver to the intestine. These disorders involve injury to cholangiocytes, inflammation, and fibrosis of the ductal system, often leading to cholestasis and impaired bile flow. The term encompasses a broad range of congenital and acquired conditions.

Cholangiopathies can be congenital or acquired. Congenital examples include biliary atresia, Alagille syndrome, and choledochal cysts. Acquired cholangiopathies include primary sclerosing cholangitis, primary biliary cholangitis, ischemic cholangiopathy (often after hepatic artery injury or transplantation), and cholangiopathy due to biliary obstruction or drug- or toxin-induced injury.

Symptoms vary but commonly include jaundice, dark urine, pale stools, pruritus, fatigue, and right upper quadrant discomfort. Laboratory tests show cholestatic patterns with elevated alkaline phosphatase and gamma-glutamyl transferase, and rising bilirubin in progressive disease.

Diagnosis combines clinical assessment with imaging—ultrasound, MRCP to visualize ducts, and ERCP or percutaneous cholangiography when intervention is needed. Liver biopsy may help in uncertain cases or to stage fibrosis. Treatment targets the underlying condition: ursodeoxycholic acid for certain cholangiopathies; endoscopic drainage for obstructive lesions; immunosuppression in autoimmune types; and liver transplantation for advanced disease or cholangiocarcinoma risk reduction in PSC.

Prognosis varies by etiology. Ischemic and advanced biliary diseases carry higher risk of liver failure; PSC markedly increases risk of cholangiocarcinoma and colorectal cancer in patients with inflammatory bowel disease. Ongoing surveillance and multidisciplinary care are common.