What is the Difference Between Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

The key difference between primary biliary cirrhosis and primary sclerosing cholangitis is that primary biliary cirrhosis is a condition characterized by damage to small intrahepatic bile ducts, while primary sclerosing cholangitis is a condition characterized by damage to medium to large extrahepatic and intrahepatic bile ducts.

Cholangiopathies are a group of liver diseases that affect cholangiocytes, which are epithelial cells that normally line the bile ducts in the liver. Primary biliary cholangitis and primary sclerosing cholangitis are the most important and prominent cholangiopathies in adults. Both these conditions are immune-mediated.

CONTENTS

1. Overview and Key Difference
2. What is Primary Biliary Cirrhosis  
3. What is Primary Sclerosing Cholangitis
4. Similarities – Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis
5. Primary Biliary Cirrhosis vs. Primary Sclerosing Cholangitis in Tabular Form
6. Summary – Primary Biliary Cirrhosis vs. Primary Sclerosing Cholangitis

What is Primary Biliary Cirrhosis?

Primary biliary cirrhosis is a chronic condition in which the bile ducts in the liver are slowly destroyed. It is characterized by damage in small intrahepatic bile ducts. Although primary biliary cirrhosis affects both sexes, it is mostly common in women. Primary biliary cirrhosis is an autoimmune disease. The symptoms of this condition may include fatigue, itchy skin, dry eyes and mouth, pain in the upper abdomen, swelling of the spleen, bone, muscle or joint pain, edema, ascites, fatty deposit development called xanthomas, jaundice, hyperpigmentation, osteoporosis, high cholesterol, diarrhea, underactive thyroid (hypothyroidism), and weight loss. Primary biliary cirrhosis occurs when T cells mistakenly destroy healthy cells lining the small bile ducts in the liver.

Primary biliary cirrhosis can be diagnosed through physical examinations, liver tests, antibody tests for signs of autoimmune disease, cholesterol tests, ultrasound tests, fibroscans, magnetic resonance cholangiopancreatography (MRCP), and magnetic resonance elastography (MRE). Treatment options for primary biliary cirrhosis are medicines such as ursodeoxycholic acid (UDCA), obeticholic acid (Ocaliva), fibrates (Tricor), budesonide, and liver transplant.

What is Primary Sclerosing Cholangitis?

Primary sclerosing cholangitis is a disease of the bile ducts specifically characterized by damage to medium to large extrahepatic and intrahepatic bile ducts. Moreover, symptoms of primary sclerosing cholangitis may include fatigue, itching, jaundice, abdominal pain, fever, chills, night sweats, enlarged liver, enlarged spleen, and weight loss. An immune system reaction to an infection or toxin may trigger primary sclerosing cholangitis in people who are genetically predisposed to this disease. The risk factors for this condition include age (most often diagnosed between the ages of 30 and 40), sex (occurs more often in men), having inflammatory bowel disease, and geographical location (people with Northern European heritage are affected more).

Primary sclerosing cholangitis can be diagnosed through physical examination, liver function blood test, MRI of bile ducts, X-rays of bile ducts, and liver biopsy. Furthermore, treatment options for primary sclerosing cholangitis may include bile acid sequestrants, antibiotics, antihistamines, opioid antagonists, ursodeoxycholic acid (UDCA), nutrition therapy, balloon dilation, stent placement, and liver transplant.

What are the Similarities Between Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis?

• Primary biliary cholangitis and primary sclerosing cholangitis are the most prominent cholangiopathies in adults.
• Both conditions are immune-mediated and progressive.
• Both conditions may have similar symptoms, such as fatigue, itchy skin, jaundice, and weight loss.
• These conditions can be diagnosed through physical examinations and imaging tests.
• They can be treated through specific medications and liver transplants.

What is the Difference Between Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis?

Primary biliary cirrhosis is characterized by damage to small intrahepatic bile ducts, while primary sclerosing cholangitis is characterized by damage to medium to large extrahepatic and intrahepatic bile ducts. Thus, this is the key difference between primary biliary cirrhosis and primary sclerosing cholangitis. Furthermore, risk factors for primary biliary cirrhosis are sex (affected women more), age (occurs more in people 30 to 60 years old), genetics, geography (common in people of northern European descent), infections, smoking, and toxic chemicals. On the other hand, risk factors for primary sclerosing cholangitis are age (most often diagnosed between the ages of 30 and 40), sex (occurs more often in men), having inflammatory bowel disease, and geographical location (people with Northern European heritage affected more).

The infographic below presents the differences between primary biliary cholangitis and primary sclerosing cholangitis in tabular form for side-by-side comparison.

Summary – Primary Biliary Cirrhosis vs. Primary Sclerosing Cholangitis

Cholangiopathies are chronic liver diseases that target the cells lining the bile ducts called the cholangiocytes. Primary biliary cholangitis and primary sclerosing cholangitis are the most prominent cholangiopathies in adults. Both these conditions primarily affect bile ducts. However, primary biliary cirrhosis is a disease of the bile ducts specifically characterized by the damage to small intrahepatic bile ducts, while primary sclerosing cholangitis is a disease of the bile ducts that is specifically characterized by the damage to medium to large extrahepatic and intrahepatic bile ducts. So, this summarizes the difference between primary biliary cirrhosis and primary sclerosing cholangitis.

Reference:

1. “Primary Biliary Cholangitis.” Mayo Clinic, Mayo Foundation for Medical Education and Research.
2.“Primary Sclerosing Cholangitis.” National Institute of Diabetes and Digestive and Kidney Diseases, U.S. Department of Health and Human Services.

Image Courtesy:

1. “Primary biliary cirrhosis intermed mag” By Nephron – Own work (CC BY-SA 3.0) via Commons Wikimedia
2. “CT of primary sclerosing cholangitis” By Dima Nimri (image) and Akshun Kalia (annotations) – CT_PSC.jpg at Wiki doc.org (CC BY-SA 3.0) via Commons Wikimedia