Primary Sclerosing Cholangitis:

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 and 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)

Diagnosis:

Primary Sclerorosis Cholangitis. Images from ERCP show alternating areas of stricture (black arrows) and dilated segments of the intrahepatic ducts (white arrows) characterictic of the “beaded” appearance seen with primary sclerosing cholangitis.
© learningradiology.com

Primary sclerosing cholangitis is diagnosed by visualizing the bile duct with imaging, usually in the setting of endoscopic retrograde cholangiopancreatography (ERCP), which shows “beading” (both strictures and dilation) of bile ducts inside and outside of the liver. Another option is magnetic resonance cholangiopancreatography (MRCP), where magnetic resonance imaging is used to visualize the biliary tract.

Most people with PSC have evidence of autoantibodies. Approximately 80% of people with PSC have perinuclear anti-neutrophil cytoplasmic antibodies; however, this finding is not specific to those with the disease. Antinuclear antibodies and anti-smooth muscle antibody are found in 20%-50% of PSC patients and, likewise, are not specific for the disease.

Other tests often done are a full blood count, liver enzymes, bilirubin levels (usually grossly elevated), kidney function, and electrolytes. Fecal fat determination is occasionally ordered when the symptoms of malabsorption are prominent.

Treatment:

Treatments for primary sclerosing cholangitis focus on managing complications and monitoring liver damage. Many medications have been studied in people with primary sclerosing cholangitis, but so far none have been found to slow or reverse the liver damage associated with this disease.

Treatment for itching

• Antihistamines, such as diphenhydramine and loratadine, are commonly used to reduce itching.
• Bile acid sequestrants (cholestyramine).

Treatment for infections

• Bile that backs up in narrowed or blocked ducts causes frequent bacterial infections. To prevent and treat these infections, people with primary sclerosing cholangitis may take repeated courses of antibiotics or continue taking antibiotics for long periods. Before any procedure that could cause an infection, such as an endoscopic procedure or abdominal surgery, you’ll also need to take antibiotics.

Nutrition support

• Primary sclerosing cholangitis makes it difficult for the body to absorb certain vitamins. Even though you may eat a healthy diet, you may find that you can’t get all the nutrients you need. Your doctor may recommend vitamin supplements that you take as tablets or that you receive as an infusion through a vein in your arm. If the disease weakens your bones, you may take calcium and vitamin D supplements as well.

Treatment for bile duct blockages

• Balloon dilation. This procedure can open blockages in the larger bile ducts outside the liver. In balloon dilation, your doctor runs a slender tube with an inflatable balloon at its tip (balloon catheter) through an endoscope and into a blocked bile duct. Once the balloon catheter is in place, the balloon is inflated.
• Stent placement. In this procedure, your doctor uses an endoscope and attached instruments to place a small plastic tube called a stent in a blocked bile duct to hold the duct open.
  Liver transplant

Ultimate treatment

• A liver transplant is the only treatment known to cure primary sclerosing cholangitis. During a liver transplant, surgeons remove your diseased liver and replace it with a healthy liver from a donor. Liver transplant is reserved for people with liver failure or other severe complications of primary sclerosing cholangitis. Though uncommon, it’s possible for primary sclerosing cholangitis to recur after a liver transplant.