Primary Biliary Cholangitis

Diagnosis

Your healthcare professional reviews your personal and family medical history and performs a physical examination. Several tests and procedures may be used to diagnose primary biliary cholangitis.

Blood tests are commonly ordered and include:

• Liver tests
  These tests measure levels of specific proteins and enzymes that can signal liver disease and injury to the bile ducts.

• Antibody tests for autoimmune disease
  Blood tests may check for anti-mitochondrial antibodies, also called AMAs. These antibodies are rarely found in people without primary biliary cholangitis, even if other liver diseases are present. Because of this, a positive AMA test is considered a strong indicator of the condition. A small number of people with the disease may not have detectable AMAs.

• Cholesterol testing
  More than half of people with primary biliary cholangitis have significantly elevated blood fats, including total cholesterol.

Imaging tests can help confirm the diagnosis or rule out other conditions with similar symptoms. Tests used to examine the liver and bile ducts may include:

• Ultrasound
  This test uses high-frequency sound waves to create images of structures inside the body.

• FibroScan
  Using an ultrasound-like probe, this test measures liver stiffness and can detect liver scarring.

• Magnetic resonance cholangiopancreatography
  Often called MRCP, this specialized MRI produces detailed images of the bile ducts and surrounding organs.

• Magnetic resonance elastography
  Also known as MRE, this test combines MRI with sound waves to develop an image called an elastogram. It helps detect liver stiffness that may point to cirrhosis.

If test results do not clearly confirm the diagnosis, a liver biopsy may be performed. During this procedure, a small sample of liver tissue is removed with a thin needle and examined in a laboratory to confirm the diagnosis or assess how advanced the disease is.

Treatment

Treating the disease

There is no cure for primary biliary cholangitis, but medications can slow disease progression and help prevent complications.

Treatment options may include:

• Ursodeoxycholic acid
  Also called UDCA or ursodiol, this medicine is usually the first treatment prescribed. It helps bile flow through the liver, improves liver function and slows scarring. It does not cure the disease and may not relieve symptoms such as fatigue or itching. Possible side effects include weight gain, hair loss and diarrhea.

• Fibrates
  Medicines such as Tricor may reduce liver inflammation and itching when used with UDCA. The exact way these drugs work is not fully understood, and more studies are needed to confirm long-term benefits.

• Budesonide
  This corticosteroid may offer benefit when combined with UDCA in certain cases. However, it can cause steroid-related side effects, particularly in people with advanced disease. Additional long-term research is needed before it can be routinely recommended.

• Liver transplant
  If medications no longer control the disease and liver failure develops, a liver transplant may be considered. Transplantation often leads to excellent long-term outcomes, although the disease can return years later in some people.

Treating symptoms

Your healthcare team may recommend treatments aimed at easing symptoms and improving quality of life.

Treatment for fatigue
Fatigue is common in primary biliary cholangitis. Daily habits, diet, exercise and other medical conditions can influence how severe fatigue feels. Testing for thyroid disease is important, as it is more common in people with this condition.

Treatment for itching
Several options may help control itching, including:

• Antihistamines, such as diphenhydramine, hydroxyzine or loratadine, which may also improve sleep.

• Cholestyramine, a powder mixed with food or liquid that can reduce itching.

• Rifampin, an antibiotic that may decrease itching by altering how the brain processes itch-related signals.

• Opioid antagonists containing naloxone or naltrexone, which can reduce itching by affecting brain receptors.

• Sertraline, an SSRI that increases serotonin levels and may lessen itching.

Treatment for dry eyes and mouth
Artificial tears and saliva substitutes can help relieve dryness. Chewing gum or sucking on hard candy may also stimulate saliva production and ease dry mouth.

Treating complications

Primary biliary cholangitis can lead to several complications, which may require additional treatment.

• Vitamin and mineral supplementation
  If nutrient absorption is reduced, supplements such as vitamins A, D, E and K may be needed. Calcium, iron or folic acid supplements may also be recommended.

• Cholesterol-lowering treatment
  Statin medications may be prescribed to manage high cholesterol levels.

• Treatment for bone loss
  If osteoporosis develops, calcium, vitamin D or other medications may be used to slow bone loss and improve bone density. Regular weight-bearing exercise can also help strengthen bones.

• Management of portal hypertension
  In advanced disease, increased pressure in the portal vein may develop. Your healthcare team will monitor for this condition. Mild abdominal fluid buildup may be managed with reduced salt intake, while more severe cases may require diuretics or a procedure called paracentesis to remove excess fluid.