Cholestasis - Causes, Diagnosis, and Treatment

Introduction:

Cholestasis is a liver disease. Bile is a fluid produced by the liver that helps digest food, especially fats. Cholestasis is a condition that occurs when the bile flow from the liver is reduced or blocked. While bile flow is stopped, a pigment called bilirubin (a waste product formed when old red blood cells (RBC) are broken down) moves into the bloodstream and accumulates. Cholestasis is common in late pregnancy.

What Are the Types of Cholestasis?

There are two distinct types of cholestasis: intrahepatic cholestasis and extrahepatic cholestasis. Intrahepatic cholestasis is formed within the liver, and extrahepatic cholestasis is caused by a physical obstruction to the bile ducts.

What Are the Symptoms of Cholestasis?

Not all patients with cholestasis are symptomatic. Some adults with chronic cholestasis are asymptomatic.

Some of the symptoms of cholestasis include:

• Jaundice (yellowing of the skin and the white of the eyes).

• Dark urine.

• Clay-colored or light-colored stool.

• Nausea.

• Vomiting.

• Inability to digest certain foods.

• Fatigue.

• Excessive itching.

• Pain in the abdomen, especially in the right upper part.

What Are the Causes of Cholestasis?

There are many causes of cholestasis.

Extrahepatic Cholestasis Is Caused By:

• Bile duct tumors.

• Pancreatitis.

• Primary sclerosing cholangitis.

• Stones in the common bile duct.

• Narrowing of the bile duct.

• Cysts.

Intrahepatic Cholestasis Is Caused By:

• Diseases such as alcoholic liver disease and primary biliary cirrhosis.

• Pregnancy.

• Diseases like tuberculosis, viral hepatitis, and sarcoidosis.

• Infections.

• Lymphoma.

• Liver cancer.

• Genetic abnormalities.

• Hormonal effects on bile flow.

Medications - The liver plays an important role in metabolizing medications. Some medications are difficult to metabolize and can be harmful to the liver.

Those medications include:

• Antibiotics such as Amoxicillin.

• Non-steroidal anti-inflammatory drugs such as Ibuprofen.

• Anabolic steroids.

• Certain antiepileptic drugs.

• Certain antifungal drugs.

• Certain antimicrobial drugs.

• Certain antipsychotic drugs like Chlorpromazine.

• Oral contraceptives.

Diseases Which Cause Cholestasis Include:

• Viral infections such as hepatitis, cytomegalovirus, Epstein-Barr virus, and human immunodeficiency virus.

• Bacterial infections.

• Genetic disorders like sickle cell disease.

• Autoimmune diseases like primary biliary cirrhosis.

• Liver and pancreatic cancer.

Cholestasis of Pregnancy: Intrahepatic cholestasis of pregnancy, otherwise known as obstetric cholestasis, is most common in the last trimester of pregnancy. The most common symptom is itching without a rash. Itching usually occurs on the hands and feet but can also affect the other parts of the body. The itching is often worse at night. This is caused due to buildup of bile acids in the blood.

Some other symptoms which are seen include:

• Jaundice.

• Pale stools.

• Nausea.

• Abdominal pain.

• Dark urine.

Cholestasis that develops during pregnancy can be an inherited condition. Women carrying multiple pregnancies are at higher risk of obstetric cholestasis. Pregnancy hormones may also cause this condition. Cholestasis of pregnancy is a serious condition that could affect both the mom and baby. Most cases resolve after delivery. However, women who had cholestasis during pregnancy have a three times greater risk of getting liver cancer later in life. Doctors usually prescribe medicines that reduce itching and that do not harm the baby. Cholestasis of pregnancy can sometimes result in early delivery, stillbirth, and lung problems in the newborn baby. There is no way to prevent cholestasis in pregnancy.

What Tests Are Done for the Diagnosis of Cholestasis?

A doctor may suspect cholestasis in people with jaundice and determine the cause based on the symptoms and physical examination.

The following tests are done:

• A blood test shows an increase in serum bilirubin and alkaline phosphatase levels.

• Liver function tests.

• Measurement of the level of bile acids in the blood.

Some other imaging tests which are done to confirm the diagnosis include:

• Computed tomography (CT) scan.

• Magnetic resonance imaging (MRI) scan.

• Abdominal ultrasound.

• Ultrasonography of liver and bile ducts.

• A liver biopsy is done if the cause of cholestasis is found to be within the liver.

If the cause is due to obstruction of the bile ducts, the following tests are done:

• Endoscopic Retrograde Cholangiopancreatography: A flexible tube (endoscope) is inserted through the mouth into the small intestine, and a radiopaque contrast agent is administered through the tube into the bile and pancreatic ducts, and x-rays are taken. This can clearly identify the blockages of bile ducts.

• Magnetic Resonance Cholangiopancreatography: This uses magnetic fields to look at the bile and pancreatic ducts.

• Endoscopic Ultrasonography: An ultrasound probe attached with a flexible endoscope is inserted through the mouth into the small intestine, and images are taken.

What Is the Treatment of Cholestasis?

The cause of the cholestasis should be diagnosed properly and treated accordingly.

• Removing the stones in the bile duct can treat the disease.

• If there is a narrowing of the bile ducts, placing a stent to open areas of the common bile duct can treat the symptoms.

• If a medication is causing the problem, stopping the drug can easily cure the disease.

• In obstetric cholestasis, it resolves after delivery. Women who develop cholestasis during pregnancy should be monitored post-pregnancy.

• The majority of the patients develop itching, which is possibly treated with antihistamines, Ursodeoxycholic acid, and Phenobarbital. Cholestyramine is also given. Soaking the itching areas in lukewarm water can provide some relief.

• Since there will be malabsorption of fats, a diet substituting carbohydrates and proteins for fats is recommended.

• When bilirubin starts to accumulate, a deficiency of fat-soluble vitamins can occur. So vitamin A, D, E, and K supplements are given.

Some measures to reduce the risk of cholestasis include:

• Getting vaccinated for hepatitis.

• Avoid alcohol overuse.

• Avoid drug overuse.

What Are the Complications of Cholestasis?

Some complications of the disease include:

• Diarrhea.

• Severe itching.

• Poor absorption of fats.

• Weak bones (osteomalacia) if untreated for a long time.

• Organ failure if there is sepsis.

Conclusion

Cholestasis can occur at any age, and recovery depends on the underlying cause and how well it can be managed. Early diagnosis and treatment can improve the chances of recovery. The patient can get back to normal once the bile flow is restored.