Primary sclerosing cholangitis or PSC is a disease of the bile ducts. Bile duct is responsible for carrying bile, which is a digestive liquid from the liver to the small intestine. In primary sclerosing cholangitis inflammation causes scars bile ducts and leads to obstruction of bile ducts both inside and outside of the liver. The obstruction causes by primary sclerosing cholangitis blocks the flow of bile, which can ultimately lead to liver cirrhosis, liver failure, and liver cancer.
Primary sclerosing cholangitis is believed to be due to autoimmunity. Majority of patients with Primary sclerosing cholangitis also have ulcerative colitis. Liver transplant is the ultimate treatment for Primary sclerosing cholangitis, typically about 10 years after being diagnosed with the disease. PSC may also lead to bile duct cancer. Endoscopy and MRI tests may be done to monitor the disease.
Signs and Symptoms:
Primary sclerosing cholangitis is characterized by recurrent episodes of cholangitis (inflammation of the bile ducts), with progressive biliary scarring and obstruction.
Early signs and symptoms:
— Chronic fatigue (a non-specific symptom often present in liver disease)
— Pruritus (itching)
Many people diagnosed with primary sclerosing cholangitis before they have symptoms continue to feel generally well for several years, but there’s no reliable way to predict how quickly or slowly the disease will progress for any individual. Signs and symptoms that may appear as the disease progresses include:
— Malabsorption (especially of fat) and steatorrhea (fatty stool) due to biliary obstruction, leading to decreased levels of the fat-soluble vitamins, A, D, E & K.
— Signs of cirrhosis
— Hepatomegaly (enlarged liver)
— Severe jaundice
— Portal hypertension
— Ascending cholangitis, or infection of the bile duct.
— Dark urine due to excess conjugated bilirubin, which is water soluble, being excreted by the kidneys
— Hepatic encephalopathy (confusion caused by liver dysfunction)