Primary biliary cholangitis

Diagnosis

To diagnose primary biliary cholangitis, your physician will inquire about your medical history and your family’s medical history, and carry out a physical examination. The subsequent examinations and procedures might be utilized for diagnosis.

Blood tests:

• Liver tests. These blood examinations look at the levels of enzymes that could indicate liver illness and bile duct damage.
• Autoimmune disease antibody test. An examination of anti-mitochondrial antibodies (AMAs) through blood tests can be conducted to determine the presence of the disease. In general, individuals without the disease are highly unlikely to have these antibodies, even if they suffer from other liver conditions. As a result, a positive AMA test is generally regarded as a dependable indication of the disease. However, a small percentage of individuals with primary biliary cirrhosis may not exhibit AMAs.
• Cholesterol test. Primary biliary cholangitis causes severe elevations in blood lipids (fats), including total cholesterol levels in more than half of affected individuals.

Your doctor may consider ordering imaging tests to confirm the diagnosis or eliminate other conditions that have similar symptoms. These tests may focus on examining the liver and bile ducts and could be helpful in determining the course of treatment.

• Ultrasound. Ultrasound creates images of the inside structures of your body using high-frequency sound waves.
• FibroScan. This test can find liver scarring by using a probe that resembles an ultrasound.
• Magnetic Resonance Cholangiopancreatography (MRCP). Your organs and bile ducts are visualized in great detail during this specialized Magnetic Resonance Imaging (MRI) test.
• Magnetic Resonance Elastography (MRE). To produce an elastogram, which is a visual depiction of internal organs, sound waves and MRI are combined. The test is used to find liver fibrosis, which may be an indicator of cirrhosis.

In cases where the diagnosis remains unclear, a liver biopsy may be conducted by your doctor. This involves the extraction of a small portion of liver tissue through a minor incision using a thin needle. The obtained tissue sample will then undergo laboratory analysis to either verify the diagnosis or determine the disease’s stage and severity.

Treatment

Primary biliary cholangitis has no known cure, however there are drugs that can help slow the disease’s course and minimize complications. Options consist of:

• Ursodeoxycholic Acid (UDCA). The medication referred to as ursodiol (Actigall, Urso) is frequently the initial treatment used. Its primary function is to facilitate the passage of bile through the liver. Although it does not provide a cure for primary biliary cholangitis, it has been shown to enhance liver function and decrease liver scarring. However, it may not be as effective in alleviating symptoms such as itching and fatigue. Side effects may include diarrhea, hair loss, and weight gain.
• Obeticholic acid (Ocaliva). Back in 2016, the U.S. Food and Drug Administration granted approval for this particular medication to be utilized in the treatment of primary biliary cholangitis. Research studies have revealed that administering this medication either independently or in combination with ursodiol over a period of 12 months can lead to improved liver function and a deceleration in liver fibrosis. Nonetheless, its usage is frequently restricted due to the potential side effect of heightened itchiness.
• Fibrates (Tricor). The exact mechanism by which this medication reduces the symptoms of primary biliary cholangitis is unknown. Yet, some persons have reported a reduction in liver irritation and inflammation when combined with UDCA. Long-term advantages require further research.
• Budesonide. The corticosteroid budesonide may be advantageous for primary biliary cholangitis when coupled with UDCA. For those with more severe condition, this medication has steroid-related adverse effects. Before budesonide may be advised for the treatment of this disorder, further extensive long-term trials are required.
• Liver transplant. In cases where primary biliary cholangitis becomes resistant to medication and liver failure occurs, a liver transplant may be recommended as a means of extending one’s lifespan. This involves replacing the diseased liver with a healthy one from a donor. For individuals with primary biliary cholangitis, liver transplantation is known to have highly positive long-term outcomes. Nonetheless, there is a chance that the disease may recur in the transplanted liver after several years.

Treating the symptoms

In order to relieve your discomfort and manage the primary biliary cholangitis symptoms, your doctor may suggest some therapies.

Treatment for fatigue

Fatigue is a common symptom of primary biliary cholangitis, but other factors such as your daily habits, diet, exercise routine, and overall health can also impact your energy levels. It is crucial to undergo testing to rule out the possibility of thyroid disease, especially as it is more prevalent among individuals with primary biliary cholangitis.

Treatment for itching

• Antihistamines. Itching relief medications include loratadine, diphenhydramine, and hydroxyzine hydrochloride. If itching keeps you awake, these might aid in helping you sleep.
• Cholestyramine. A powder that calls for food or liquid blending.
• Rifampin. An anti-itch medication that might work. Its exact mechanism is unknown. According to researchers, it might suppress the brain’s reaction to blood-borne molecules that cause itching.
• Sertraline. A selective serotonin reuptake inhibitor (SSRI), which can assist in easing the itching’s effects.
• Opioid antagonists. There may be relief for liver disease-related itching with medications such those that contain naltrexone and naloxone. These medications appear to function on your brain, similar to rifampin, to lessen the itching feeling.

Treatment for dry eyes and mouth

Artificial saliva and tears, which are sold over-the-counter or on prescription, can aid with dry mouth and eyes. Moreover, chewing gum or sucking on hard candies might increase saliva production and alleviate dry mouth.

Treating the complications

Primary biliary cholangitis frequently comes with a few consequences. Your physician might advise:

• Vitamin and mineral supplements. You may need to take supplements of vitamins A, D, E, and K, calcium, folic acid, or iron if your body isn’t absorbing vitamins or other nutrients.
• Medication to lower cholesterol. A drug called a statin may be prescribed by your doctor if your blood cholesterol level is high.
• Medications to treat bone loss. You may be provided medication or supplements, such as calcium and vitamin D, if you have osteoporosis, which causes weak or thinning bones, in order to improve bone density and lessen bone loss. Your bone density can be increased by exercising regularly, such as walking and lifting small weights most days of the week.
• Portal hypertension treatment. If you have more severe scarring (fibrosis or cirrhosis) from liver disease, your doctor is likely to check and monitor you for portal hypertension and enlarged veins. One common side effect of portal hypertension is the accumulation of fluid in your abdomen (ascites). If there is only slight fluid buildup, your doctor might recommend cutting back on salt in your diet. In more severe cases, medications such as diuretics or a treatment to drain the fluid (paracentesis) may be necessary.