Overview
Autoimmune hepatitis type 1 (AIH-1) is a chronic liver disease where the body's immune system mistakenly attacks the liver. Instead of protecting the body, immune cells treat liver tissue as a threat and cause ongoing inflammation. Over time, this inflammation can scar the liver — a process called fibrosis — and if left untreated, may lead to cirrhosis (severe scarring) or liver failure. AIH-1 is also sometimes called classic autoimmune hepatitis or lupoid hepatitis, an older term that is no longer preferred. The disease can affect people of any age, but it is most commonly diagnosed in women and girls. Symptoms can range from mild fatigue and yellowing of the skin to more serious signs of liver damage. Many people have no symptoms at first, and the disease is discovered through routine blood tests showing abnormal liver enzymes. The good news is that AIH-1 responds well to treatment in most people. Medications that calm the immune system — called immunosuppressants — can control the disease and prevent further liver damage. With proper treatment and regular monitoring, many patients live full, healthy lives. However, lifelong treatment and follow-up are usually needed to keep the disease under control.
Also known as:
Key symptoms:
Persistent tiredness or fatigueYellowing of the skin or whites of the eyes (jaundice)Abdominal pain or discomfort, especially in the upper right sideNausea or loss of appetiteDark-colored urinePale or clay-colored stoolsJoint pain or achingSkin rashesSwollen abdomen due to fluid buildup (ascites) in advanced diseaseEasy bruising or bleedingItchy skinFeeling 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 Autoimmune hepatitis type 1.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Autoimmune hepatitis type 1 at this time.
New trials open frequently. Follow this disease to get notified.
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 1.
Community
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Start the conversation →Latest news about Autoimmune hepatitis type 1
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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 has any scarring already occurred?,What are the expected side effects of prednisone and azathioprine, and how will we manage them?,How will we know if the treatment is working, and what does remission look like for me?,Is it possible to eventually stop medication, and what are the risks of trying?,Are there any foods, supplements, or over-the-counter medications I should avoid?,Should I be tested for other autoimmune conditions, since AIH-1 can occur alongside them?,How often do I need blood tests and liver monitoring, and what warning signs should prompt me to call you right away?
Common questions about Autoimmune hepatitis type 1
What is Autoimmune hepatitis type 1?
Autoimmune hepatitis type 1 (AIH-1) is a chronic liver disease where the body's immune system mistakenly attacks the liver. Instead of protecting the body, immune cells treat liver tissue as a threat and cause ongoing inflammation. Over time, this inflammation can scar the liver — a process called fibrosis — and if left untreated, may lead to cirrhosis (severe scarring) or liver failure. AIH-1 is also sometimes called classic autoimmune hepatitis or lupoid hepatitis, an older term that is no longer preferred. The disease can affect people of any age, but it is most commonly diagnosed in women
How is Autoimmune hepatitis type 1 inherited?
Autoimmune hepatitis type 1 follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Autoimmune hepatitis type 1?
1 specialists and care centers treating Autoimmune hepatitis type 1 are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.