Overview
Seronegative autoimmune hepatitis (also sometimes called seronegative AIH) is a form of autoimmune hepatitis where the body's immune system mistakenly attacks the liver. In typical autoimmune hepatitis, doctors can find specific proteins in the blood called autoantibodies that confirm the immune system is misfiring. In the seronegative form, those usual blood markers are absent or undetectable, which makes diagnosis more challenging — but the liver damage and symptoms are very real and can be just as serious. The liver becomes inflamed over time, which can interfere with its ability to filter toxins, produce proteins, and support digestion. If left untreated, this ongoing inflammation can lead to scarring of the liver (called fibrosis or cirrhosis) and, in severe cases, liver failure. People with this condition may feel persistently tired, notice yellowing of the skin or eyes (jaundice), have abdominal discomfort, or experience nausea and joint pain. Treatment usually involves medicines that calm down the immune system, such as corticosteroids (like prednisone) and azathioprine. These do not cure the disease but can control it effectively in many people. Some patients achieve remission and may be able to reduce their medications over time, while others need long-term treatment. In cases where the liver is severely damaged, a liver transplant may be considered. Regular monitoring by a liver specialist (hepatologist) is essential to manage this condition well.
Key symptoms:
Persistent tiredness and low energyYellowing of the skin or whites of the eyes (jaundice)Pain or discomfort in the upper right side of the abdomenNausea or vomitingLoss of appetiteJoint pain or achingDark-colored urinePale or clay-colored stoolsItchy skinSwelling of the abdomen due to fluid buildup (ascites)Easy bruising or bleedingUnexplained weight lossFeeling generally unwell or run down
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 Seronegative autoimmune hepatitis.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Seronegative autoimmune hepatitis at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for Seronegative autoimmune hepatitis.
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 Seronegative autoimmune hepatitis.
Community
No community posts yet. Be the first to share your experience with Seronegative autoimmune hepatitis.
Start the conversation →Latest news about Seronegative autoimmune hepatitis
No recent news articles for Seronegative autoimmune hepatitis.
Follow this condition to be notified when news becomes available.
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 confident are you in the diagnosis of seronegative autoimmune hepatitis, and what other conditions have been ruled out?,What does my liver biopsy show, and how much damage has already occurred?,What is the treatment plan, and how will we know if it is working?,What are the risks and side effects of the medications you are recommending?,How often will I need blood tests and follow-up appointments?,Are there any lifestyle changes — such as diet or exercise — that could help protect my liver?,At what point would a liver transplant be considered, and how do I get referred to a transplant center if needed?
Common questions about Seronegative autoimmune hepatitis
What is Seronegative autoimmune hepatitis?
Seronegative autoimmune hepatitis (also sometimes called seronegative AIH) is a form of autoimmune hepatitis where the body's immune system mistakenly attacks the liver. In typical autoimmune hepatitis, doctors can find specific proteins in the blood called autoantibodies that confirm the immune system is misfiring. In the seronegative form, those usual blood markers are absent or undetectable, which makes diagnosis more challenging — but the liver damage and symptoms are very real and can be just as serious. The liver becomes inflamed over time, which can interfere with its ability to filter
How is Seronegative autoimmune hepatitis inherited?
Seronegative autoimmune hepatitis follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.