Primary Sclerosing Cholangitis

Primary sclerosing cholangitis is a disease characterized by inflammation, thickening, and the formation of scar tissue (fibrosis) in the bile ducts. If left untreated, primary sclerosing cholangitis (PSC) can cause liver damage .

The bile ducts are the ducts that carry bile from the liver to the intestines and gallbladder. Bile plays an important role in helping the process of digesting food.

In PSC, the bile ducts become inflamed and form scar tissue. This condition will make the bile ducts narrow so that bile accumulates and triggers severe liver damage.

PSC generally develops gradually. In advanced stages, this condition can cause recurrent liver infections, cirrhosis , and liver failure.

Causes of Primary Sclerosing Cholangitis

It's not known exactly what causes primary sclerosing cholangitis . However, this condition is thought to be caused by disorders of the immune system, genetic disorders, injuries to the bile ducts, or bacterial or viral infections.

Although the cause is unknown, there are several factors that can increase a person's risk of suffering from primary sclerosing cholangitis , namely:

Aged between 30–50 years
Male gender
Suffering from inflammatory bowel disease ( IBD)
Have a family member who suffers from PSC
Suffering from gallstones
Suffer from an autoimmune disease, such as celiac disease and thyroid disease

Symptoms of Primary Sclerosing Cholangitis

Primary sclerosing cholangitis develops slowly. In its early stages, the symptoms of PSC are similar to several other diseases, so the sufferer often doesn't realize it. These symptoms include:

The body feels tired
Itchy skin
Fever
Loss of appetite
Pain in the upper right abdomen

Over time, the symptoms will get worse. Usually, this happens because primary sclerosing cholangitis has caused liver problems.

Symptoms at this stage can include:

Weight loss drastically
Frequent sweating at night
Difficulty thinking, confused, or lost memory
Easy bruising, nosebleeds, vomiting blood , or bloody bowel movements
A red rash appears on the palms and soles for no apparent reason
Swelling in the legs
Yellow eyes and skin ( jaundice )

When to see a doctor

Check with your doctor if you experience symptoms of primary sclerosing cholangitis as mentioned above. You also need to see a doctor immediately if you feel itching and your body feels tired all the time.

Primary sclerosing cholangitis is often associated with inflammatory bowel disease, such as ulcerative colitis or Crohn's disease . If you experience this condition, carry out routine controls to the doctor to monitor the condition and progress of the disease.

Diagnosis of primary sclerosing cholangitis

To diagnose primary sclerosing cholangitis , the doctor will conduct a question and answer regarding the patient's and family's complaints and medical history. After that, the doctor will do a physical examination, especially in the area of the stomach, legs and skin.

To confirm the diagnosis, the doctor will carry out the following supporting tests:

Blood test , to see the number of blood cells and detect liver function disorders
MRI scan , to see the condition of the liver and bile ducts
Endoscopic retrograde cholangiopancreatography ( ERCP ), to see the condition of the glands and bile ducts, especially if the patient has metal implants that cannot undergo an MRI
Liver biopsy , to detect liver damage by taking a sample of liver tissue and examining it in the laboratory

Treatment of Primary Sclerosing Cholangitis

There is no treatment to cure primary sclerosing cholangitis . However, treatment can be done to relieve complaints, prevent liver damage from continuing, and prevent complications.

Several treatment options that can be done in primary sclerosing cholangitis are:

Drugs

Administration of drugs in primary sclerosing cholangitis aims to reduce complaints of itching and treat infection.

Several types of drugs that can be given to reduce itching are antihistamines , UDCA ( ursodeoxycholic acid ), bile acid sequestrants, and creams or lotions containing camphor , menthol, pramoxine , and capsaicin .

Antibiotic drugs will also be given to patients with primary sclerosing cholangitis who are at risk of developing an infection in the liver due to narrowing and blockage in the bile ducts.

Operation

Several types of operations that can be performed on primary sclerosing cholangitis are:

Installation of a balloon catheter, to open blockages in the bile ducts outside the liver
Ring installation ( stent ), to keep the bile ducts open
Liver transplantation , to replace damaged liver tissue in PSC patients with liver failure

The balloon catheter and stent placement procedure is usually performed when the patient is undergoing ERCP. Meanwhile, liver transplantation is done by open surgery ( laparotomy ).

Supportive therapy

Primary sclerosing cholangitis is prone to causing malnutrition because the body has difficulty absorbing certain vitamins and minerals. To treat this condition, the doctor will prescribe supplements and vitamins.

Although rare, primary sclerosing cholangitis liver can sometimes recur after a transplant procedure. Therefore, patients need to carry out routine controls to the doctor to monitor their condition.

Complications of Primary Sclerosing Cholangitis

Complications that can occur due to primary sclerosing cholangitis are:

Liver disease and liver failure
Recurrent liver infections
Portal hypertension, due to high blood pressure (hypertension) in the veins in the liver (portal vein)
Osteoporosis
Bile duct cancer ( cholangiocarcinoma )
Colon cancer

Prevention of Primary Sclerosing Cholangitis

Primary sclerosing cholangitis is hard to prevent, but if you have a condition such as IBD or an autoimmune disease, have regular check-ups with your doctor to monitor your condition and progress.

If you are already experiencing PCS, take the following steps to prevent further liver damage: