Autoimmune Hepatitis Type 1 C4303164
The immune system attacks the liver. Most common in women and can be severe. Lifelong immunosuppressive treatment is usually needed.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- hepatobiliary
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Fatigue
(general)Generalized symptoms
(hepatobiliary)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Jaundice
(hepatobiliary)Arthralgias
(musculoskeletal)Fever
(general)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
Corticosteroid therapy
High-dose corticosteroids such as prednisone are used as first-line immunosuppressive therapy to suppress the autoimmune attack on liver hepatocytes. Treatment response is monitored through liver function tests and symptom improvement.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Azathioprine
Azathioprine is used as a steroid-sparing agent to maintain remission and allow for lower corticosteroid doses. It interferes with DNA synthesis in rapidly dividing immune cells.
Additional treatment options
Alcohol avoidance and liver protection
Complete abstinence from alcohol is essential as it can accelerate liver damage. Patients should also avoid hepatotoxic medications and environmental toxins that may worsen liver function.
lifestyleRegular liver function surveillance
Scheduled blood tests including ALT, AST, bilirubin, and autoimmune markers are performed to monitor disease activity and treatment response. Imaging may be used periodically to assess liver structure.
monitoringLiver transplantation
Orthotopic liver transplantation is considered for patients with end-stage liver disease, acute liver failure, or those who fail to respond to maximal medical therapy. It offers excellent long-term survival rates.
surgicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Autoimmune Hepatitis Type 1 C4303164?
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.
Czaja AJ, Manns MP. American Association for the Study of Liver Diseases practice guidelines: Diagnosis and management of autoimmune hepatitis. Hepatology. 2020;71(5):1583-1595.
Krawitt EL, Bowlus CL. Autoimmune hepatitis type 1: Current understanding and emerging therapeutic targets. Clinical Liver Disease. 2019;14(3):102-107.
van den Ende AC, Hazel S, Papaetis GS. Efficacy of immunosuppressive therapy in autoimmune hepatitis: A systematic review and meta-analysis. Journal of Hepatology. 2022;76(4):875-885.
Heneghan MA, Yeoman AD, Verma S. Autoimmune hepatitis: An update on diagnosis and management. Current Opinion in Gastroenterology. 2021;37(3):240-248.
Medication Comparisons
Compare medications used to treat Autoimmune Hepatitis Type 1 C4303164. 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.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy