Primary biliary cholangitis (PBC) is a chronic autoimmune disease that damages the bile ducts in the liver leading to a harmful buildup of bile.
It primarily impacts women over 40 and can progress to liver failure if untreated. Understanding the common signs, diagnosis, treatment options, and lifestyle factors can help patients effectively manage this condition.
What Is Primary Biliary Cholangitis?
Primary biliary cholangitis (PBC) is a chronic autoimmune disease characterized by progressive destruction of the small bile ducts within the liver.
This leads to cholestasis, or impaired bile flow, resulting in a harmful buildup of bile acids and other toxins.
Symptoms Of Primary Biliary Cholangitis
The most characteristic PBC symptoms include:
▪️ Fatigue
Defeating fatigue and a general lack of energy as a result of the liver’s diminished ability to digest sugars and proteins.
▪️ Itching
The buildup of bile salts can cause many sections of the skin, including the hands, feet, and other areas of the body, to experience a painful itching sensation.
▪️ Jaundice
As bilirubin pigment accumulates, there is a yellowing of the skin as well as the whites of the eyes.
▪️ Abdominal discomfort
The inflammation and expansion of the liver can cause symptoms such as bloating, cramps, and pain in the upper right abdominal region.
▪️ High cholesterol
A decrease in the function of the liver might lead to an increase in LDL and triglycerides.
Other possible symptoms are dark urine, pale stool, bone loss, thyroid problems, and an enlarged spleen. Many patients are asymptomatic early on or have mild nonspecific symptoms, so blood tests are key for diagnosis.
Treatment Of Primary Biliary Cholangitis
While there is no cure for PBC, the first-line medication is ursodeoxycholic acid (UDCA) to improve bile flow. Additional therapies include:
▪️ Ursodeoxycholic Acid (UDCA) –
This medication is the first-line treatment for PBC. UDCA helps improve bile flow and reduce liver inflammation and scarring. It has been shown to slow disease progression and improve survival.
▪️ Obeticholic Acid
For patients who don’t respond adequately to UDCA, this medication can be added. Obeticholic acid further improves bile flow and liver function.
▪️ Cholestyramine
This bile acid-binding resin can help relieve itching symptoms but does not treat the underlying disease.
▪️ Vitamin Replacement
Supplements of fat-soluble vitamins A, D, E, and K are often needed as malabsorption is common with PBC. Calcium and vitamin D supplementation is also recommended.
▪️ Liver Transplant
For patients with end-stage PBC who develop liver failure, a liver transplant may be necessary. This provides normal bile flow again.
▪️ Lifestyle Changes
Avoiding other liver toxins can help preserve liver function. This includes restricting alcohol intake and certain medications that stress the liver.
▪️ Treatment of Complications –
Associated conditions like osteoporosis, thyroid disease, and vitamin deficiencies should also be treated properly.
▪️ Monitoring
Ongoing follow-up is key, with bloodwork to assess response to therapy and screen for complications. Liver imaging may be used to track structural changes.
Alongside medications, avoiding potential liver toxins like alcohol is recommended. Monitoring for osteoporosis with bone density scans is also advised. Supportive care from a hepatologist is key.
Primary Biliary Cholangitis and Diet
No specific diet is universally recommended, but some dietary adjustments may help:
| Low fat | Decreases demands on the liver to metabolize fats. |
| Limit sodium | Prevents fluid retention that stresses liver. |
| Calcium and vitamin D | Support bone health. |
| Limit sugar | Prevents fluid retention that stresses the liver. |
| No supplements | Can stress the liver. Get nutrients from food instead. |
Maintaining adequate nutrition with fruits, vegetables, lean proteins, and whole grains is important, as PBC increases calorie requirements. A registered dietitian can provide guidance.
Diagnosis of Primary Biliary Cholangitis
PBC diagnosis involves:
➜ Physical exam – Assesses liver enlargement and jaundice.
➜ Blood tests – Checks liver enzymes (ALK phosphatase) and antibodies (ANA and anti-mitochondrial antibodies).
➜ Imaging – Ultrasound or MRI to visualize liver abnormalities.
➜ Liver biopsy – Examines bile duct inflammation but is often not required for diagnosis.
Based on test results, PBC is staged from early stage 1 (mild) to stage 4 (cirrhosis). Accurate staging helps guide treatment and predict the prognosis.
Frequently Asked Questions
The exact cause is unknown but it involves autoimmune destruction of the bile ducts. Genetic and environmental factors likely contribute.
With treatment, most patients live a normal lifespan. 10-15% eventually require a liver transplant.
In PSC, the bile ducts become scarred and narrowed. PBC causes immune-mediated bile duct destruction.
There is no known cure yet, but medications paired with lifestyle modifications can help slow progression and manage symptoms.
Summary
PBC arises when the body’s immune system attacks bile duct cells leading to progressive liver damage.
Early diagnosis, proper treatment, and healthy lifestyle habits are key to preserving liver function and quality of life.
Patients should have regular monitoring with both medical and holistic support.
