Primary Sclerosing Cholangitis – Life Expectancy, Symptoms, Prognosis

Primary sclerosing cholangitis (PSC) is a chronic disease condition that affects the liver. Before we go into details, we will describe some of the liver functions. One vital function of the liver is to manufacture bile. Bile, which is vital in the digestion process, is to aid in the digestion of fat and the elimination of toxins from the body.  When the bile ducts are affected, the disorder is given the medical term primary sclerosing cholangitis.

Source – radiologyassistant.nl

PSC, which is a medical condition that affects adults, is characterized by inflammation and fibrosis that affect intrahepatic and extrahepatic bile ducts, causing bile duct obliteration and cirrhosis as the disease progresses.

Sclerosing is a medical term which means the scarring and hardening of bile ducts due to the presence of chronic inflammation, while cholangitis is a medical term which means bile duct inflammation. When not  treated properly, the condition eventually lead to liver failure.

Primary Sclerosing Cholangitis Symptoms

When a person experience this disease in the early stage, he or she will encounter no symptoms at all. Yet, some people, unfortunately, have reported to experience PSC symptoms such as the following:

• Hepatomegaly or enlargement of the liver
• Ascending cholangitis or infection of the bile duct
• Hepatic encephalopathy
• Portal hypertension
• Cirrhosis or scarring of the liver
• Fat Malabsorption
• Steatorrhea
• Weight loss
• Severe jaundice
• Intense pruritus or intense itching
• Chronic fatigue
• Upper abdominal pain
• Fever
• Chills

Primary Sclerosing Cholangitis Causes

The specific cause of PSC is still not known. According to statistics, most of the reported cases of this disease occur in men. According to research, the disease condition exists due to the following:

• Presence of viral infection
• Presence of bacterial infection
• Immune system disorder
• Genetic factors

A study was conducted, and it showed that people who acquired PSC are those who have other medical conditions such as inflammatory bowel disease. The link between these two diseases is still undergoing study.

Primary Sclerosing Cholangitis Pathophysiology

Due to the presence of inflammation, PSC  causes the distruction of the bile ducts both inside and outside the liver. The inflammation will cause scarred bile ducts to hinder the flow of bile, leading to cholestatis, a condition where the bile cannot flow normally from the liver to the duodenum.  The presence of bile stasis and the back flow pressure lead to the induction of epithelial cell proliferation and the destruction of the parenchyma of the liver, causing formation of bile lakes. When there is a chronic form of biliary obstruction, portal tract fibrosis and biliary cirrhosis occur, and eventually, in a worse-comes-to-worst case scenario, liver failure. When a person has cholestatis, there will be fat malabsorption as well as fat soluble vitamin deficiencies, which include those of vitamin A, D, E,and K.

Primary Sclerosing Cholangitis Diagnosis

As mentioned earlier, people with PSC most often have no symptoms. The only way to confirm that they have this disease is through diagnostic tests like the following:

• Liver function blood exam

It is an exam which tests the function of the liver, including the liver enzyme level.

• Magnetic resonance cholangiopancreatography

It is a non-invasive exam that gives a clear picture of the bile ducts, pancreas, and liver.

• Liver biopsy
  

A portion of the liver is culled, and a sample of the tissue is examined under the microscope which is done in the laboratory.

• Cholangiography

It is an X-ray exam that examines the bile ducts through the use of a dye which is injected through a needle or  an endoscope.

• Endoscopic retrograde cholangiopancreatography

It is an exam that uses a combination of fluoroscopy and endoscopy to diagnose as well as treat the problems of the pancreatic ductal or biliary systems.

• Bilirubin level exam

This is an exam which indicates the level of bilirubin. People with PSC will show a grossly elevated Bilirubin level.

• Renal function test

This is an exam which indicates the state of the kidneye and the person’s renal physiological state.

• Fecal fat determination test

It is an exam or test that determines the condition of the fat malabsorption, possibly causing steatorrhea (excessive fat in the feces).

Primary Sclerosing Cholangitis Treatment and Diet

The good news about PSC is that it is can be treated symptomatically through the following treatments:

Pharmacological treatment

Medications that are often prescribed to patients with PSC are as follows:

Treatment for itching

• Bile acid drugs such as cholestyramine
• Antihistamine

Treatment for infection

• Antibiotics

Ttreatment for malnutrition

• Multivitamins
• Vitamin supplements

Surgical treatment

Depending on the degree of the disease, the surgeon will determine the type of surgical procedure that needs to be done. The common surgical procedures that are performed are as follows:

• Bile duct surgical procedure
• Balloon dilation and placement of stent
• Liver transplantation

Diet modification

Since the target of PSC is the liver, particularly the production of bile, it is advised that the affected person avoids fat-rich foods and consumes raw vegetables and fruits. Protein can be obtained from fresh fish or organic poultry. Furthermore, it is also recommended that the person avoids alcoholic beverages.

Primary Sclerosing Cholangitis Prognosis

The prognosis of people with PSC varies. According to studies, the expected life span of people who have this disease condition is approximately 25 years. Moreover, compared to those who experience symptoms, people who experience no symptoms have a better outlook. Another study that was conducted showed that one out of ten is at risk of  having bile duct cancer and colon cancer. Furthermore, it has been estimated that the survival is about 12 years from the diagnosis in PSC patients. Prolongation of life then depends on the need for liver transplantation and the response of the body towards the given treatment.