Primary Sclerosing Cholangitis: Symptoms, Treatment
Overview
Primary sclerosing cholangitis (PSC) is a chronic disease of bile duct. If you have this condition, it means your bile ducts inside and outside the liver have become inflamed and scarred, and are finally narrowed or even blocked. This will make bile build up in the liver and lead to liver damage. Although primary sclerosing cholangitis progresses slowly, it can cause cirrhosis, liver failure, repeated infections, and tumors of the bile duct or liver.
It is estimated that about 6 to 16 people out of 100,000 have primary sclerosing cholangitis. Considering that many people with the condition haven’t been diagnosed, the actual number should be higher.
Symptoms
Common signs or symptoms of PSC are:
- Fatigue
- Itchy skin
Sometimes people with PSC may have other symptoms such as:
- Losing weight without trying
- Poor appetite
- Fever
- Pain in the abdomen
As the condition progresses, symptoms that may occur include:
- Bloating
- Bruising and bleeding easily
- Confusion, difficulty thinking, or memory loss
- Redness in the palms of your hands
- Swelling in your legs, ankles, or feet
- Yellowish eyes and skin, called jaundice
Causes
Experts don’t know what exactly cause primary sclerosing cholangitis. But it is considered that the following factors may trigger the disease:
- Genes
- Immune system problems
- Changes in the bacteria in your gastrointestinal tract
- Bile duct injury caused by bile acids
Risk factors
There are some factors that may increase your risk of getting this condition.
- Age. People aging 30 to 50 are most likely to have PSC, though it can occur at any age.
- Sex. PSC is more common in men.
- Inflammatory bowel disease. According to survey, a large number of people with PSC also have inflammatory bowel disease.
- Geography. People with Northern European ancestry are more often diagnosed with PSC.
Complications
Primary sclerosing cholangitis may cause complications such as:
- Liver disease and failure
- Repeated infections
- Portal hypertension
- Thinning bones
- Bile duct cancer
- Colon cancer
Diagnosis
Your doctor may use the tests and procedures below to make a diagnosis of primary sclerosing cholangitis:
- Liver function blood tests
- MRI of your bile ducts
- X-rays of your bile ducts
- Liver biopsy
Treatment
There is no cure for primary sclerosing cholangitis. But doctors can use treatment to manage complications and monitor liver damage.
Treatment for itchy skin
- Bile acid sequestrants
- Antibiotics
- Antihistamines
- Opioid antagonists
- Ursodeoxycholic acid (UDCA)
Treatment for infections
Accumulation of bile in narrowed or blocked ducts will lead to frequent bacterial infections. In order to prevent and treat these infections, doctors may recommend antibiotics for long periods.
Nutrition support
Since your it is difficult for you body to absorb vitamins as normal because of PSC, you may need vitamin supplements which you take as tablets or receive as an infusion through a vein in the arm. Calcium and vitamin D will be used if it weakens your bones.
Treatment for bile duct blockages
Your doctor may determine the cause of blockages with endoscopic retrograde cholangiopancreatography (ERCP) and treat it with:
- Balloon dilation
- Stent placement
Liver transplant
At present, a liver transplant is the only treatment that can cure PSC. However, it’s likely that PSC recur after a transplant. This option is reserved for those who have liver failure or other severe complications.
Keywords: primary sclerosing cholangitis; PSC.
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