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Symptoms, Causes, Diagnosis, and Treatment

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Symptoms, Causes, Diagnosis, and Treatment

Although there is no cure for primary biliary cholangitis, there are several treatment options that help slow disease progression as well as manage symptoms and complications.

Medications to Treat Primary Biliary Cholangitis

These drugs work to help slow the progression of primary biliary cholangitis and often help relieve symptoms as well.

Ursodiol (Actigall) is a commonly used medication containing ursodeoxycholic acid (UDCA), which helps protect the liver by reducing the amount of bile acids produced by the liver. It is a first-line treatment for slowing liver damage. The dosage is typically 13 to 15 milligrams per kilogram (mg/kg) of body weight, taken twice daily. UDCA can slow liver disease progression in many patients.

Ursodiol is considered the gold standard of PBC treatment. People who don’t respond to UDCA may be placed on additional medications, typically after one year of treatment.

Obeticholic acid (Ocaliva) is a bile acid modulator that reduces bile acid production and increases bile flow. It’s used in people who don’t respond well to UDCA or as an add-on therapy. Dosing starts with 5 milligrams (mg) once daily, increasing to 10 mg depending on tolerance and liver disease stage. The drug may improve liver biochemistry and reduce fibrosis progression.

At the end of 2024, the FDA issued a warning regarding the risk of serious liver injury in patients taking obeticholic acid who did not have cirrhosis of the liver. Such injury had already been seen and warned about in patients with cirrhosis of the liver.

In anyone taking obeticholic acid, liver function should be monitored frequently, and the treatment should be stopped if signs of liver deterioration appear. Patients are advised to discuss this updated safety information with their doctors, and to weigh the risks and benefits of continuing obeticholic acid or exploring other treatment options.

Elafibranor (Iqirvo) is a peroxisome proliferator-activated receptor (PPAR)–alpha and PPAR-delta agonist. It’s used alone or in combination with UDCA as an option for second-line PBC therapy in people without decompensated cirrhosis who were intolerant to UDCA or had an inadequate response. The dose is 80 mg once daily. The drug helps control liver inflammation and the levels of certain substances such as bile acids.

Seladelpar (Livdelzi) is a selective peroxisome proliferator-activated receptor-delta (PPARδ) agonist. It’s used alone or in combination with UDCA as an option for second-line PBC therapy in people without decompensated cirrhosis who were intolerant to UDCA or had an inadequate response. The dosage is 10 mg once daily. The drug helps lower levels of alkaline phosphatase (ALP), a liver enzyme and key indicator of PBC, and helps relieve itching.

Fibrates (fenofibrate, bezafibrate)
modulate bile acid metabolism and have anti-inflammatory effects.
They’re used off label in patients with incomplete response to UDCA. Fibrates are not used in people with decompensated cirrhosis. Fibrates may improve liver function tests and itching.

Symptom Management for Primary Biliary Cholangitis

These medications can help relieve symptoms, especially itching. Most are not specifically approved to treat PBC but rather are used off label.

Bile acid sequestrants (cholestyramine)
bind bile acids in the gut, reducing itching. The dosage is 4 g one to four times daily, depending on severity. These may interfere with the absorption of other medications.

 


Rifampin (Rimactane) is an antibiotic that affects bile acid metabolism, reducing itching.
It is a second-line treatment for itching if cholestyramine is ineffective. The dosage is 150 to 300 mg twice daily.

Naltrexone
is an opioid receptor antagonist that reduces central itch signaling. It is a third-line treatment for itching. The dosage is 25 to 50 mg once daily.

Sertraline is a selective serotonin reuptake inhibitor (SSRI) that increases serotonin in the brain. It helps reduce itching. The dosage is 50 to 100 mg per day.

Gabapentin reduces the release of excitatory neurotransmitters in the brain, helping to reduce itching. The dosage is 100 to 3,600 mg per day

Antihistamines, including over-the-counter antihistamines such as diphenhydramine (Benadryl) and loratadine (Alavert, Claritin), may help with itching.

Topical lotions may help with itchy skin. Products that contain menthol or cooling agents can be especially effective.

Medical Procedures

There is evidence that ultraviolet B (UVB) phototherapy may help relieve itching.

Immunizations

Unless blood serum indicates evidence of immunity, people with PBC should be given vaccinations for hepatitis A and hepatitis B.

Supportive Medications

Vitamin and mineral supplements
are used to address deficiencies due to malabsorption (fat-soluble vitamins A, D, E, K, and calcium), especially if serum bilirubin is greater than 2mg/dL. They can also prevent complications like osteoporosis.

Bisphosphonates (alendronate)
can be used to prevent osteoporosis if osteopenia is present, or treat it in patients with PBC.

Surgery: Liver Transplantation


If liver function continues to decline and symptoms are unresponsive to medications, a person may be put on the liver transplant list. Liver transplant surgery has excellent outcomes for people with PBC.

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