Date published: July 7, 2023
Autoimmune and
Cholestatic Liver Disease
Overview, Diagnosis and Treatment Options
Autoimmune liver diseases occur when the body’s immune system mistakenly attacks liver cells, leading to inflammation.
If left untreated, this inflammation can progress to cirrhosis, significantly increasing the risk of liver cancer and ultimately leading to liver failure.
What are the most common types?
There are three primary types of autoimmune and cholestatic liver diseases.
Autoimmune Hepatitis (AIH)
This condition arises when the immune system attacks liver cells, often without any noticeable symptoms until significant liver damage has occurred. AIH can affect individuals of all ages, including children, and may progress to cirrhosis or acute liver failure if untreated.
Primary Biliary Cirrhosis (PBC)
In PBC, immune cells target and destroy the bile ducts within the liver, leading to bile flow obstruction. Symptoms may include itching, dry eyes, dry mouth, fatigue, bone disease, and elevated cholesterol, though many individuals remain asymptomatic. Without intervention, PBC can advance to cirrhosis and liver failure.
Primary Sclerosing Cholangitis (PSC)
Known to cause inflammation and scarring of the bile ducts which can prevent bile from passing through. Consistent with other autoimmune liver diseases, symptoms could be nonexistent or linked to symptoms like itching or jaundice. A frequent association is found with IBS (inflammatory bowel disease). Complications of PSC involve cancers of the liver, bile duct and gallbladder in addition to biliary infections and cirrhosis.
Both PBC and PSC are the most common chronic cholestatic liver diseases in adults, linked to a condition known as cholestasis, where bile flow from the liver is reduced or blocked. Cholestasis has both intrahepatic (within the liver) and extrahepatic (outside the liver) causes, and if untreated, it can lead to fibrosis, biliary cirrhosis, and ultimately, end-stage liver disease.
How do we diagnose autoimmune & cholestatic liver diseases?
Diagnosing autoimmune liver diseases can be challenging, as these conditions often present complex and overlapping symptoms leading to frequent misdiagnoses or missed diagnoses altogether.
Early symptoms are often subtle or absent, and patients may not experience noticeable issues until the disease has progressed to cirrhosis or liver failure.
At Methodist Transplant Specialists, a multidisciplinary team provides an advanced, expert-driven approach to both diagnosis and treatment.
Initial diagnosis typically begins with blood tests, which can reveal markers associated with autoimmune liver conditions.
Additionally, diagnostic imaging and, if needed, a liver biopsy may be performed to confirm the diagnosis and assess disease severity.
How do we treat autoimmune and cholestatic liver diseases?
Autoimmune Hepatitis Treatment
Treatment focuses on suppressing the immune system to reduce liver inflammation.
Options include:
Immunosuppressive Therapy: Standard treatment includes corticosteroids (e.g., prednisone) often combined with azathioprine to control immune response and reduce inflammation.
Biologics and Advanced Therapies: For patients unresponsive to conventional treatments, options may include biologic agents targeting specific immune pathways.
Primary Biliary Cholangitis Treatment
PBC therapy aims to enhance bile flow and prevent further liver damage.
Options include:
Ursodeoxycholic Acid (UDCA): The first-line treatment for improving bile flow and reducing liver damage.
Seladelpar: A recently approved medication recommended for patients with an inadequate response to UDCA, aimed at slowing the progression of liver disease.
Symptomatic Relief: Additional medications may be prescribed to alleviate symptoms, such as pruritus (itching).
Primary Sclerosing Cholangitis (PSC) Treatment
Ongoing clinical trials are investigating new therapies, as options remain limited.
Options include:
Liver Transplant: The only definitive treatment for advanced PSC, although it is reserved for patients with end-stage liver disease.
Experimental Therapies: New treatments, such as antifibrotic drugs and bile acid therapies, are under investigation but currently lack FDA approval.
Advanced Autoimmune Liver Disease Treatment
In severe cases, such as end-stage liver disease, a liver transplant may be necessary.
Autoimmune and cholestatic liver diseases are chronic and progressive, often requiring long-term medical management and, in some cases, liver transplantation.
Early diagnosis and treatment are crucial to manage symptoms, reduce liver damage, and improve patient outcomes.
Our team at Methodist Transplant Specialists carefully reviews all medical and clinical histories, diagnostic results, and other relevant information to ensure an accurate diagnosis.
This information has been medically reviewed by Mangesh R. Pagadala, MD, Transplant Hepatologist & Gastroenterologist
If you have questions or would like to speak with our team, please call us at 214.947.4400 or 1.877.4A.LIVER.