Autoimmune Hepatitis Type 2 C4303163
A more aggressive form of liver autoimmunity, often affecting children and young adults. Requires long-term immune suppression.
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)Abdominal pain
(hepatobiliary)Loss of appetite
(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
Intensive corticosteroid therapy
Type 2 AIH often requires more aggressive initial treatment with higher dose corticosteroids due to its typically more severe presentation with anti-LKM1 antibodies. Response rates are generally favorable with appropriate dosing.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Azathioprine combination therapy
Combined with corticosteroids as first-line maintenance therapy. Azathioprine helps achieve steroid-free remission and reduces long-term corticosteroid complications.
Additional treatment options
Mycophenolate mofetil
An alternative immunosuppressant for patients who are intolerant to azathioprine or fail to achieve remission. It inhibits inosine monophosphate dehydrogenase in lymphocytes.
medicationHepatic diet modification
Sodium restriction and adequate protein intake with careful caloric balance support liver function. Small frequent meals may help with nausea and maintain nutrition in patients with decreased hepatic synthetic function.
dietaryLiver transplantation
Reserved for acute liver failure or progression to cirrhosis despite optimal medical management. Type 2 AIH may have higher relapse rates post-transplant requiring vigilant monitoring.
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 2 C4303163?
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.
Mack CL, Adams D, Assis DN, et al. Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance From the American Association for the Study of Liver Diseases. Hepatology. 2020;71(1):255-273.
Strassburg CP, Manns MP. Autoimmune hepatitis type 2: anti-LKM antibodies and clinical aspects. J Hepatol. 2019;71(5):1031-1042.
Zhang WC, Zhao Q, Chen B. Efficacy and Safety of Immunosuppressive Therapy for Autoimmune Hepatitis Type 2: A Systematic Review and Meta-Analysis. Clin Gastroenterol Hepatol. 2021;19(11):2341-2353.
Greene MC, Hupertz VB, Bove KE. Pediatric Autoimmune Hepatitis Type 2: Clinical Features and Long-Term Outcomes. J Pediatr Gastroenterol Nutr. 2022;74(3):412-420.
Medication Comparisons
Compare medications used to treat Autoimmune Hepatitis Type 2 C4303163. 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