primary biliary cholangitis

Primary Biliary Cholangitis (PBC): A Chronic Liver Disease

Primary biliary cholangitis, previously known as primary biliary cirrhosis, is a chronic liver disease that affects the small bile ducts in the liver. The condition is characterized by the progressive destruction of these ducts, leading to inflammation and scarring.

Key Features:

• Autoimmune Nature: PBC is an autoimmune disease, where the immune system mistakenly attacks the liver's bile ducts.
• Chronic Progression: The disease progresses slowly over time, with symptoms worsening gradually.
• Affects Women: PBC mostly affects middle-aged women, with a mean age of 39 years. Onset usually occurs in persons aged 30-65 years [5].
• Liver Damage: The destruction of bile ducts leads to liver damage and scarring.

Symptoms:

While symptoms may vary from person to person, common signs of PBC include:

• Fatigue
• Itching (pruritus)
• Jaundice (yellowing of the skin and eyes)
• Dark urine
• Pale stools

References:

• [1] Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed.
• [2] Primary biliary cholangitis is a chronic disease in which the small bile ducts in the liver become injured and inflamed and are eventually destroyed.
• [3] Primary Biliary Cholangitis (PBC) is a chronic liver disease resulting from the progressive destruction of the bile ducts in the liver.
• [4] Primary biliary cholangitis (PBC, often referred to as primary biliary cirrhosis) is a type of liver disease that can get gradually worse over time.

Common Signs and Symptoms of Primary Biliary Cholangitis

Primary biliary cholangitis (PBC) can manifest in different ways, and not everyone experiences the same symptoms. However, there are some common signs and symptoms that are often associated with this condition.

• Fatigue: Feeling extremely tired is one of the most common symptoms of PBC, affecting about 65% of people with the disease [1].
• Itchy skin (pruritus): Itching in any part of the body can be a symptom of PBC, and it's reported by around 55% of people with the condition [2].
• Discomfort or pain in the upper-right side of the abdomen: Some people may experience discomfort or pain in this area due to liver inflammation [3].
• Joint pain or arthritis: Joint pain or arthritis can occur in some individuals, often accompanied by other symptoms like dry eyes and dry mouth [4].
• Dry eyes and dry mouth (Sjögren's syndrome): PBC is associated with Sjögren's syndrome, a condition that causes dryness in the eyes and mouth [5].

Other possible symptoms

In addition to these common symptoms, some people may experience:

• Yellowing of the skin and eyes (jaundice)
• Pain in the upper right abdomen
• Bone and joint aches
• Fatigue (extreme tiredness)

It's essential to note that not everyone with PBC will exhibit all of these symptoms. In fact, many people have no symptoms at all when they are first diagnosed [6]. If you're experiencing any of these symptoms or suspect you may have PBC, consult a healthcare professional for proper evaluation and treatment.

References:

[1] Context result 3 [2] Context result 5 [3] Context result 9 [4] Context result 13 [5] Context result 8 [6] Context result 4

Diagnostic Tests for Primary Biliary Cholangitis

Primary biliary cholangitis (PBC) is a type of liver disease that can be challenging to diagnose, but various tests and procedures can help confirm the condition. Here are some diagnostic tests used to diagnose PBC:

• Blood Tests: Blood tests are often the first step in diagnosing PBC. They may include:
  • Liver tests: To check for elevated levels of liver enzymes, such as alanine transaminase (ALT) and aspartate transaminase (AST).
  • Cholesterol tests: People with PBC may have higher-than-normal cholesterol levels.
  • Antimitochondrial antibody (AMA) test: This test checks for the presence of AMA, which is a common marker for PBC. [8][9]
• Imaging Tests: Imaging tests can help rule out other causes of bile duct damage and confirm the diagnosis of PBC. These may include:
  • X-rays
  • Ultrasounds
• Liver Biopsy: A liver biopsy involves removing a small sample of liver tissue with a needle and sending it to a lab for examination under a microscope. This test can help confirm the diagnosis of PBC by showing damage to the bile ducts. [10]
• Enzyme-linked immunosorbent assay (ELISA) tests: ELISA tests are highly sensitive and specific for PBC, with a sensitivity of 95% and specificity of 98%. However, false-positive results can occur in autoimmune hepatitis (type 1). [13]

It's essential to note that the diagnosis of PBC is often based on a combination of these tests and procedures, as well as a thorough medical history and physical examination. A healthcare provider will use this information to determine the best course of action for diagnosing and treating PBC.

References:

[8] Anti-mitochondrial antibody (AMA) blood test - Up to 95% of patients with PBC will have a positive AMA test. [9] Find out about primary biliary cholangitis (primary biliary cirrhosis), which is a type of long-term liver disease where the bile ducts in the liver become damaged. [10] February 18, 2024 - Your doctor may remove a small sample of your liver tissue with a needle and send it to a lab. This test is usually done at a hospital or medical center, so you can get medicine to make you sleepy and control pain. [13] Enzyme-linked immunosorbent assay (ELISA) tests are 95% sensitive and 98% specific for PBC; false-positive results can occur in autoimmune hepatitis (type 1).

Medications Used to Treat Primary Biliary Cholangitis

Primary biliary cholangitis (PBC) is a type of liver disease that damages the bile ducts, and early recognition and treatment can help prevent complications. Medications play a crucial role in managing PBC.

• Ursodeoxycholic acid (UDCA): Also known as ursodiol, UDCA is a natural bile acid that helps move bile out of the liver and into the small intestine. It's the main drug used to treat PBC and can slow down the progression of liver damage [10][14].
• Obeticholic acid (OCA): OCA works by improving bile flow and reducing inflammation in the liver. It's another FDA-approved treatment for PBC, which can be used alongside UDCA or as an alternative [8][9][13].
• Prednisone or azathioprine: These medications are prescribed to suppress the immune system in patients with the "overlap syndrome" who have autoimmune hepatitis along with PBC [4].

Other Potential Treatments

Research is ongoing to find new treatments for PBC. For example, an international clinical trial involving UC Davis Health suggests that Elafibranor may be a potential novel treatment for PBC, showing significant improvements in biomarkers of the disease [5]. Additionally, antihistamines are first-line agents to relieve pruritus (itching) in early stages, and cholestyramine and colestipol can sequester bile salts [6].

Important Considerations

It's essential for patients with PBC to work closely with their healthcare providers to determine the best treatment plan. This may involve monitoring liver function tests, adjusting medication dosages as needed, and addressing any side effects or complications that arise.

References: [1] November 14, 2023 - Primary biliary cholangitis is a type of liver disease that damages the bile ducts. [2] May 1, 2024 - Quit smoking, drinking alcohol ... using drugs and medications. [3] December 21, 2023 - Doctors treat primary biliary cholangitis with medicines. [4] March 22, 2024 - Medications to suppress the immune system may also be prescribed including prednisone or azathioprine in Primary Biliary Cholangitis (PBC) patients with the “overlap syndrome” with autoimmune hepatitis. [5] November 16, 2023 - An international clinical trial involving UC Davis Health suggests the investigational drug Elafibranor is a potential novel treatment for patients with the rare, autoimmune disease primary biliary cholangitis. [6] Aug 16, 2024 — Antihistamines are first-line agents to relieve pruritus in early stages. [7] Compare risks and benefits of common medications used for Primary Biliary Cholangitis . [8] Obeticholic acid (OCA) is a treatment that works by improving bile flow and reducing inflammation. [9] by RB Bernal · 2023 · Cited by 7 — Currently, there are only 2 Food and Drug Administration (FDA)-approved treatments for PBC: UDCA and OCA. [10] Obeticholic acid for the treatment of primary biliary cholangitis. [11] November 14, 2023 - Primary biliary cholangitis is a type of liver disease that damages the bile ducts. [12] Two medicines, ursodeoxycholic acid (UDCA) and obeticholic acid (OCA), can be used to treat PBC. Both medicines help move bile out of the liver into the small intestine. [13] AB Currently, the only first-line therapy for primary biliary cholangitis (PBC) approved by the US Food and Drug Administration (FDA) is ursodeoxycholic acid. This treatment is weight-based; current guidelines recommend starting at a dose of 13 to 15 mg/kg, divided in 2 to 3 doses per day. [14] July 23, 2020 - UpToDate, the evidence-based clinical decision support resource from Wolters Kluwer, is trusted at the point of care by clinicians worldwide.

Differential Diagnoses for Primary Biliary Cholangitis (PBC)

Primary biliary cholangitis (PBC) is a chronic liver disease characterized by the progressive destruction of the bile ducts in the liver. When diagnosing PBC, it's essential to consider various differential diagnoses that may cause similar symptoms and histological findings.

Key Differential Diagnoses:

• Autoimmune Hepatitis: This condition involves inflammation of the liver due to an autoimmune response, which can lead to similar liver damage as PBC [1][2].
• Primary Sclerosing Cholangitis (PSC): PSC is a disease that causes scarring and narrowing of the bile ducts, leading to cholestasis and potentially cirrhosis [3][4].
• Biliary Obstruction or Stricture: Conditions such as gallstones, tumors, or strictures can cause obstruction of the bile ducts, mimicking the symptoms of PBC [2][5].
• Cholangiocarcinoma: This is a type of cancer that affects the bile ducts and can present with similar symptoms to PBC, including jaundice and abdominal pain [6].

Other Differential Diagnoses:

• Extrahepatic or intrahepatic cholestasis with various etiologies
• Drug/medication-induced hepatotoxicity
• Graft Versus Host Disease (GVHD)
• Primary Sclerosing Cholangitis

It's crucial to consider these differential diagnoses when evaluating patients for PBC, as accurate diagnosis and treatment can significantly impact patient outcomes.

References:

[1] Jan 31, 2024 - Differential diagnoses of PBC may include such hepatic and systemic conditions as biliary obstruction or stricture, primary sclerosing ...

[2] Apr 4, 2021 - The main histologic differential diagnosis for PBC includes primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH), drug/medication-...

[3] Jul 29, 2020 - Primary biliary cirrhosis must be differentiated from other disease that may cause cholestasis including autoimmune hepatitis, common bile duct...

[4] Nov 21, 2019 - The most important differential diagnosis is from cholangiocarcinoma. CA 19-9 levels above 130 U/mL may suggest the diagnosis of ...

[5] by H You · 2023 · Cited by 10 — Differential diagnosis. The differential diagnosis of PBC includes extrahepatic or intrahepatic cholestasis with various etiologies.

[6] Mar 22, 2024 - Primary Biliary Cholangitis (PBC) is a chronic liver disease resulting from the progressive destruction of the bile ducts in the liver.