From the Guidelines
Autoimmune diseases that can cause liver failure include autoimmune hepatitis, primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC), with autoimmune hepatitis being the most direct cause, as indicated by recent guidelines 1. Autoimmune hepatitis is characterized by the immune system attacking liver cells, leading to inflammation, scarring, and potential liver failure if left untreated. Some key points to consider in the management of these conditions include:
- Treatment typically involves corticosteroids like prednisone and immunosuppressants such as azathioprine, which are usually continued long-term to prevent relapse.
- PBC affects the small bile ducts and is treated with ursodeoxycholic acid, while PSC affects larger bile ducts and has limited treatment options beyond managing complications.
- Overlap syndromes can also occur, combining features of multiple autoimmune liver diseases.
- Early diagnosis through blood tests and liver biopsy is crucial for effective management, as these conditions can progress to cirrhosis and liver failure without proper treatment, potentially requiring liver transplantation in advanced cases.
- The autoimmune nature of these diseases stems from a dysregulated immune response where the body's defense mechanisms mistakenly target liver tissues, often triggered by genetic predisposition combined with environmental factors, as discussed in various studies 1. The most recent and highest quality study 1 emphasizes the importance of early corticosteroid therapy in patients with acute severe autoimmune hepatitis, and considers liver transplantation in cases of severe coagulopathy and hepatic encephalopathy.
From the Research
Autoimmune Diseases Causing Liver Failure
The following autoimmune diseases can cause liver failure:
- Autoimmune Hepatitis (AIH) 2, 3, 4, 5, 6
- Primary Biliary Cirrhosis (PBC) 2, 3, 4, 5, 6
- Primary Sclerosing Cholangitis (PSC) 2, 3, 4, 5, 6
- Overlap syndromes of these three disease entities 2, 3, 5
Characteristics of Autoimmune Liver Diseases
These diseases are characterized by:
- Chronic inflammatory disorders 6
- Autoimmune attack on hepatocytes, biliary epithelial cells, or the entire biliary system 4, 5
- Elevated liver function tests 2
- Presence of specific autoantibodies, such as antimitochondrial autoantibodies in PBC 5
Diagnosis and Treatment
Diagnosis is often difficult, but can be facilitated by:
- Sequential measurement of relevant autoantibodies 2
- Exclusion of other liver diseases 2, 5
- Ultrasound, ERCP, and liver histology 2
- Liver biopsy, currently the gold standard technique for diagnosing cirrhosis 4 Treatment options include:
- Immunosuppressive therapy for AIH 2, 5
- Ursodesoxycholic acid (UDCA) for PBC 2, 5
- Mechanical endoscopic manipulation of the bile ducts, treatment of cholangitis, and UDCA for PSC 5
- Liver transplantation for end-stage liver disease secondary to AIH, PBC, or PSC 4, 5