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primary biliary cholangitis 5
Description
Primary Biliary Cholangitis (PBC) Description
Primary biliary cholangitis, often referred to as primary biliary cirrhosis, is a type of liver disease that can get gradually worse over time. This chronic condition affects the liver and can lead to serious complications if left untreated.
Key Characteristics:
- A progressive liver disorder
- Mostly affects women
- Can appear at any age, but usually appears in middle-aged individuals
- Gradually destroys intrahepatic bile ducts, leading to periportal inflammation and cholestasis
Symptoms and Progression:
PBC can cause a range of symptoms, including fatigue, itching, and jaundice. If left untreated, the condition can progress to cirrhosis and portal hypertension, which can lead to liver failure.
References:
- [5] Primary biliary cholangitis (PBC, often referred to as primary biliary cirrhosis) is a type of liver disease that can get gradually worse over time.
- [10] Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disorder that gradually destroys intrahepatic bile ducts, resulting in periportal inflammation and cholestasis.
Additional Characteristics
- Primary biliary cholangitis, often referred to as primary biliary cirrhosis, is a type of liver disease that can get gradually worse over time.
- Primarily affects the liver and can lead to serious complications if left untreated.
- Gradually destroys intrahepatic bile ducts, leading to periportal inflammation and cholestasis.
- Can cause a range of symptoms, including fatigue, itching, and jaundice.
- If left untreated, the condition can progress to cirrhosis and portal hypertension, which can lead to liver failure.
Signs and Symptoms
Common Signs and Symptoms of Primary Biliary Cholangitis
Primary biliary cholangitis (PBC) can manifest in various ways, and identifying the symptoms is crucial for early diagnosis and treatment. Here are some common signs and symptoms associated with PBC:
- Nausea and belly pain: Many people experience nausea and abdominal discomfort due to liver inflammation.
- Fatigue and loss of energy: Feeling tired and exhausted is a common symptom, as the liver's ability to filter toxins and produce energy is compromised.
- Fatty deposits under the skin: In some cases, fatty deposits can appear under the skin, particularly on the arms, legs, or buttocks.
- Fatty stools: Changes in bowel movements, such as fatty stools, may also occur due to impaired liver function.
- Itching: Itchy skin (pruritus) is a common symptom, often accompanied by dryness and irritation.
- Poor appetite and weight loss: As the disease progresses, people with PBC may experience a decrease in appetite and subsequent weight loss.
These symptoms can vary from person to person, and some individuals may not exhibit any noticeable signs at all. If you're experiencing any of these symptoms or have concerns about your liver health, consult a healthcare professional for proper evaluation and guidance.
References: * [1] Symptoms · Nausea and belly pain · Fatigue and loss of energy · Fatty deposits under the skin · Fatty stools · Itching · Poor appetite and weight loss. As liver function declines, these symptoms can become more pronounced. * [5] Cited by 4 — Symptoms · Nausea and belly pain · Fatigue and loss of energy · Fatty deposits under the skin · Fatty stools · Itching · Poor appetite and weight loss. As liver ...
Additional Symptoms
- Poor appetite and weight loss
- Nausea and belly pain
- Fatigue and loss of energy
- Fatty deposits under the skin
- Fatty stools
- itching
Diagnostic Tests
Diagnostic Tests for Primary Biliary Cholangitis
Primary biliary cholangitis (PBC) can be established if two of three objective criteria are present, including serum antimitochondrial antibody (AMA) at titers ≥ 1:40, unexplained elevated alkaline phosphatase (ALP) ≥ 1.5 times the upper limit of normal, and unexplained elevated gamma-glutamyl transferase (GGT) ≥ 2 times the upper limit of normal [5].
Key Diagnostic Tests:
- Serum AMA blood test: Up to 95% of patients with PBC will have a positive AMA test [8].
- Liver function tests: Blood tests that check the levels of certain enzymes, such as ALP and AST, to see how the liver is working [9].
- Imaging tests: X-rays and ultrasounds may be used to help rule out other causes of bile duct damage.
Other Relevant Tests:
- Bilirubin, ALP, AST, albumin, and platelet count tests can be used at baseline and for ongoing monitoring [4].
- Ultrasound scan: To help rule out other problems with the bile ducts.
- Blood tests to check for elevated cholesterol levels, which may be present in people with PBC.
References: [5] CL Bowlus · 2014 · Cited by 219 [8] Anti-mitochondrial antibody (AMA) blood test - Up to 95% of patients with PBC will have a positive AMA test. [9] Feb 18, 2024 — Blood Tests. Your doctor will want to do liver function tests to see how the organ is working.
Additional Diagnostic Tests
- Liver function tests
- Imaging tests
- Serum AMA blood test
Treatment
Recommended Medications
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Differential Diagnosis
Primary Biliary Cholangitis (PBC) Differential Diagnosis
The diagnosis of Primary Biliary Cholangitis (PBC) is based on the presence of at least 2 of 3 key criteria, including:
- Persistent elevation of serum alkaline phosphatase: This is a crucial indicator that helps distinguish PBC from other liver diseases [5].
- Presence of antimitochondrial antibodies: Most patients with PBC have these antibodies, which are used as a diagnostic marker [9].
- Histological features: The liver biopsy findings in PBC include bile duct destruction and inflammation, which can be distinguished from other conditions such as primary sclerosing cholangitis (PSC) or autoimmune hepatitis (AIH) [6].
Other Conditions to Consider
When diagnosing PBC, it's essential to rule out other conditions that may cause similar symptoms. These include:
- Primary Sclerosing Cholangitis (PSC): This is a chronic liver disease characterized by inflammation and scarring of the bile ducts.
- Autoimmune Hepatitis (AIH): This is an inflammatory condition where the immune system attacks the liver cells.
- Drug-Induced Liver Injury: Certain medications can cause liver damage, which may be mistaken for PBC.
References
[5] CL Bowlus. Primary Biliary Cirrhosis. In: Feldman M, Friedman LS, Brandt LJ, editors. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 10th ed. Philadelphia, PA: Elsevier; 2014.[number]
[9] GM Hirschfield. Primary Biliary Cholangitis. In: Rakel RE, editor. Conn's Current Therapy. 1st ed. Philadelphia, PA: Elsevier; 2018.[number]
[6] H You. Primary Biliary Cholangitis. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023.[number]
Additional Differential Diagnoses
- Drug-Induced Liver Injury
- primary sclerosing cholangitis
- autoimmune hepatitis
Additional Information
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- A primary biliary cholangitis that has_material_basis_in variation at the ZPBP2 locus on chromosome 17q12-q21.
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Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.
It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.