Primary Biliary Cholangitis 4 C3280201
A form of autoimmune liver disease targeting bile ducts. It predominantly affects women and can cause scarring of the liver. Early treatment helps manage symptoms and slow disease progression.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- hepatobiliary
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Ascites
Generalized symptoms
(hepatobiliary)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Itchy skin
(hepatobiliary)Right upper quadrant discomfort
(hepatobiliary)Spider angiomata
(hepatobiliary)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Ursodeoxycholic acid (UDCA)
First-line bile acid therapy that helps protect liver cells and improve bile flow. This treatment is the cornerstone of PBC management and has been shown to slow disease progression in most patients.
Alcohol avoidance and liver protection
Complete abstinence from alcohol and avoidance of hepatotoxic substances. A healthy lifestyle supports liver function and prevents additional injury to hepatic tissue.
Additional treatment options
Obeticholic acid (second-line)
An alternative or adjunctive bile acid therapy for patients who respond inadequately to UDCA alone. This FXR agonist helps reduce bile acid toxicity and inflammation in liver tissue.
medicationLow-sodium, balanced nutrition
Dietary modification to reduce sodium intake and maintain adequate protein consumption. Proper nutrition helps manage ascites and supports overall liver function.
dietaryRegular hepatic function surveillance
Scheduled blood tests and imaging to monitor disease progression, treatment response, and development of complications such as portal hypertension or hepatocellular carcinoma.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Primary Biliary Cholangitis 4 C3280201?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Lindor KD, Bowlus CL, Boyer J, et al. Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019.
Hirschfield GM, Gershwin ME. The immunopathology of primary biliary cholangitis. Clin Liver Dis. 2022.
Nevens F, Andreone P, Mazzella G, et al. A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis. N Engl J Med. 2016.
Zhang Y, Li G, He Q, et al. Efficacy and Safety of Obeticholic Acid for Treatment of Primary Biliary Cholangitis: A Systematic Review and Meta-Analysis. J Hepatol. 2021.
Medication Comparisons
Compare medications used to treat Primary Biliary Cholangitis 4 C3280201. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy