Primary sclerosing cholangitis (bile duct disease)

Description A progressive disea...

Description

A progressive disease of the bile ducts of the liver and the ducts that drain the gallbladder. It is characterized by inflammation and scarring of the ducts. The bile ducts develop narrowed areas (strictures) causing blocked bile flow. Cirrhosis (liver scarring) and liver failure occur. Bile duct cancer may occur in up to 15% of patients. The disorder is frequently associated with inflammatory bowel disease.

Symptoms

Jaundice (yellowing skin), dark urine, abdominal pain (usually in the right upper abdomen), itching, ill feeling (malaise), loss of appetite, indigestion, enlarged liver, enlarged spleen, excess fat in stool (steatorrhea).

Tests

Common tests used for diagnosis and treatment

Workup:
A history and physical exam will be performed. Testing begins with blood tests. Other tests that may be done include: ERCP (endoscopic retrograde cholangiopancreatography), PTC (percutaneous transhepatic cholangiogram), liver biopsy, MRCP (abdominal MRI/magnetic resonance cholangiopancreatography).

Tests:
Complete blood count (CBC), Comprehensive metabolic panel (CMP), CT Scan, Lipase, Magnetic resonance imaging (MRI), Ultrasound

Other Specific Tests: ERCP (endoscopic retrograde cholangiopancreatography), PTC (percutaneous transhepatic cholangiogram), liver biopsy, MRCP (abdominal MRI/magnetic resonance cholangiopancreatography)

Specialists:
Gastroenterology, Pediatric Gastroenterology

Treatment

Large bile ducts can be kept open with an implanted "stent" if they become too narrow. This may partially relieve symptoms. Antibiotics are prescribed for co-existing infections. This disease can only be cured by liver transplantation.