Autoimmune hepatitis

Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease in which the body's immune system mistakenly attacks its own liver cells (hepatocytes), leading to progressive liver inflammation and damage. It was previously known as lupoid hepatitis or chronic active hepatitis. The disease primarily affects the liver but can be associated with other autoimmune conditions such as thyroiditis, type 1 diabetes, celiac disease, ulcerative colitis, and rheumatoid arthritis. AIH is characterized by elevated serum aminotransferases, the presence of circulating autoantibodies (such as anti-nuclear antibodies, anti-smooth muscle antibodies, or anti-liver kidney microsomal antibodies), and elevated immunoglobulin G (IgG) levels. Two main types are recognized: Type 1 (the most common, associated with ANA and SMA antibodies) and Type 2 (associated with anti-LKM1 antibodies, more common in children). Symptoms can range from mild and nonspecific — including fatigue, malaise, joint pain, nausea, and abdominal discomfort — to severe presentations with jaundice, dark urine, and signs of acute liver failure. Some patients are asymptomatic at diagnosis and are identified through incidentally discovered abnormal liver function tests. If left untreated, AIH can progress to liver fibrosis, cirrhosis, and end-stage liver disease requiring liver transplantation. The disease affects women more frequently than men, with a female-to-male ratio of approximately 3.6:1. Treatment is based on immunosuppressive therapy. First-line treatment typically involves corticosteroids (prednisone or prednisolone), often combined with azathioprine as a steroid-sparing agent. Most patients respond well to immunosuppression, achieving biochemical and histological remission, though many require long-term or lifelong maintenance therapy to prevent relapse. For patients who do not respond to standard therapy, second-line agents such as mycophenolate mofetil, tacrolimus, or cyclosporine may be considered. Liver transplantation remains an option for patients with acute liver failure or decompensated cirrhosis unresponsive to medical treatment, with generally good post-transplant outcomes, although disease recurrence in the graft can occur.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Autoimmune hepatitis