Causative and risk factors
The exact cause is not known. Primary sclerosing cholangitis may be seen in patients who have autoimmune disorders, chronic pancreatitis, inflammatory bowel disease or sarcoidosis. This condition is also common in individuals who have calculi or infections in the liver, gallbladder, and bile ducts.
Clinical presentation
The patient experiences pain in the right upper or middle portion of the upper abdomen. The pain is episodic and its intensity may vary. The pain may radiate to the back or below the right shoulder blade. Fever may be present with chills. The patient develops jaundice. Other symptoms include diarrhoea, pruritus and unintended loss of weight. Some patients may remain asymptomatic.
Primary sclerosing cholangitis can give rise to complications like liver cirrhosis, portal hypertension, cholangiocarcinoma, bile duct strictures and liver failure.
Investigations
Laboratory tests are performed to measure the white blood count and the liver function tests.
Imaging scans such as ultrasound or CT scan of the abdomen are carried out. Specialized investigations such as endoscopic retrograde cholangiopancreatography (ERCP), magnetic resonance cholangiopancreatography (MRCP) and percutaneous transhepatic cholangiogram (PTCA) are performed. Liver biopsy may be performed.
Treatment
A liver transplant is the only treatment known to cure primary sclerosing cholangitis.
Other treatment is aimed at relieving the signs and symptoms. Medications include the use of antihistamines, bile-acid binding drugs and antibiotics. ERCP is a diagnostic as well as therapeutic procedure to relieve the blockages. Surgical procedures may be needed to relieve the bile duct blockages.
