Overview
Autoimmune hepatitis type 2 (AIH-2) is a rare liver disease where the body's immune system mistakenly attacks healthy liver cells. Unlike most autoimmune liver diseases that mainly affect adults, AIH-2 most often affects children and teenagers, though adults can also develop it. It is sometimes called anti-LKM1 positive autoimmune hepatitis, named after a specific antibody found in the blood of most people with this condition. In AIH-2, the immune system produces antibodies — mainly anti-liver kidney microsomal type 1 (anti-LKM1) antibodies — that target a protein called CYP2D6 found in liver cells. This ongoing immune attack causes inflammation and, over time, can lead to scarring of the liver (cirrhosis) if left untreated. Symptoms can include yellowing of the skin and eyes (jaundice), extreme tiredness, belly pain, and a swollen abdomen. The good news is that AIH-2 responds well to treatment in most cases. Doctors typically use corticosteroids like prednisone and immune-suppressing drugs like azathioprine to calm the immune system and protect the liver. With proper treatment, many patients can live full lives, though lifelong medication and regular monitoring are usually needed. In severe cases, a liver transplant may be considered.
Also known as:
Key symptoms:
Extreme tiredness and low energyYellowing of the skin and whites of the eyes (jaundice)Pain or discomfort in the upper right bellySwollen belly due to fluid buildup (ascites)Nausea and loss of appetiteDark-colored urinePale or clay-colored stoolsItchy skinJoint painEnlarged liver or spleenUnexplained weight lossEasy bruising or bleedingSkin rashes
Multifactorial
Caused by a mix of several genes and environmental factors
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Autoimmune hepatitis type 2.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Autoimmune hepatitis type 2 at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for Autoimmune hepatitis type 2.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Autoimmune hepatitis type 2.
Community
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Start the conversation →Latest news about Autoimmune hepatitis type 2
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.How severe is my liver disease right now, and is there already any scarring?,What is the treatment plan, and how long will I need to take these medications?,What side effects should I watch for with prednisone and azathioprine?,How will we know if the treatment is working, and what does remission look like?,What are the signs of a relapse, and what should I do if I notice them?,Are there any dietary changes or supplements I should make to protect my liver?,At what point would a liver transplant be considered, and what does that process involve?
Common questions about Autoimmune hepatitis type 2
What is Autoimmune hepatitis type 2?
Autoimmune hepatitis type 2 (AIH-2) is a rare liver disease where the body's immune system mistakenly attacks healthy liver cells. Unlike most autoimmune liver diseases that mainly affect adults, AIH-2 most often affects children and teenagers, though adults can also develop it. It is sometimes called anti-LKM1 positive autoimmune hepatitis, named after a specific antibody found in the blood of most people with this condition. In AIH-2, the immune system produces antibodies — mainly anti-liver kidney microsomal type 1 (anti-LKM1) antibodies — that target a protein called CYP2D6 found in liver
How is Autoimmune hepatitis type 2 inherited?
Autoimmune hepatitis type 2 follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.