Autoimmune Liver Disease
What is Autoimmune Hepatitis?
Autoimmune hepatitis (AIH) is a rare and chronic auto-immune liver disease where the immune system attacks your liver cells.
Your liver makes many proteins, is good at clearing toxins, and is key for a healthy immune system. In AIH however, your immune cells attack the liver and this injury causes inflammation (hepatitis), which if not treated results in liver damage and scarring (i.e., cirrhosis).
AIH is a lifelong disease that can be managed by working together with your medical team to use the best treatments to control the inflammation.
How do I know I have AIH?
Many patients with AIH will not have symptoms. If symptoms exist, they may include:
Aches/pains in muscles
Jaundice (yellowing of the skin and eyes.)
How is AIH Diagnosed?
Currently, there is no one perfect test for AIH. Instead, a blood test is used to see if patients with AIH have the following symptoms:
Presence of Auto-antibodies (e.g. ANA, SMA)
Higher amounts of IgG (a type of antibody that fights infections)
High liver enzymes, particularly ALT or AST.
Diagnosis is also based on making sure there is no infection, no medication related liver damage, and nothing has been inherited. A liver biopsy is frequently used to help confirm the diagnosis and the need for long-term treatment.
Why do I have AIH?
Every year probably over 1000 people in Canada are diagnosed with AIH. It occurs in people of all ages, ethnicities, and genders. Although the cause is unknown, AIH may be triggered by environmental toxins in people who have an underlying genetic predisposition. These environmental toxins may include: infections, drugs, herbs bacteria in the bowel, and other substances we have yet to be discovered.
People with AIH will often have other autoimmune diseases such as thyroid disease, celiac disease, or rheumatoid arthritis.
How is AIH Treated?
Treatments are tailored to each individual patient, as AIH is a disease that differs between each person. AIH is however managed well by suppressing the immune system and decreasing inflammation in the liver. This is achieved first through corticosteroids (working like a fire-extinguisher to rapidly treat inflammation), then maintained with drugs like azathioprine (like a smoke-detector to stop inflammation coming back). Treatment has been shown to make people live longer.
AIH patient pamphlet download:
Primary Biliary Cholangitis
What is Primary Biliary Cholangitis?
The liver is responsible for creating bile, which aids in the process of digestion and the removal of waste and drugs from the body. Bile is carried from the liver to gallbladder by the small bile ducts. Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease where the immune system attacks the small bile ducts in the liver.
Over time bile may not leave the liver as well as it should and this retained bile causes an inflammatory response by the immune system leading to bile duct damage and destruction. Over time, if the inflammation from the immune system and retained bile is left untreated, then liver damage accumulates and patients can develop cirrhosis.
How do I know I have PBC?
Many individuals with PBC will not have symptoms.
If symptoms are present, the most common ones include:
Dryness (eyes, mouth, vagina)
Restless legs at night
How is PBC Diagnosed?
PBC is usually diagnosed with blood tests, and it is less common to need a liver biopsy nowadays.
Patients with PBC present with higher values of the liver enzyme alkaline phosphatase (ALP) in their blood. PBC diagnosis is then confirmed when antimitochondrial antibodies (AMAs) are detected in blood tests. When an ultrasound is performed, it is usually normal.
Approximately 10% of patients with PBC will be negative for AMA in their blood. When this occurs and PBC is suspected, further blood tests can help make the diagnosis, or a liver biopsy may be needed.
Many patients now also get a Fibroscan at diagnosis, and then every following year. This ultrasound based test is good for measuring liver inflammation and scarring.
How is PBC Treated?
The current initial treatment for PBC is a bile acid called Ursodeoxycholic Acid (UDCA).
UDCA works by stimulating bile secretion, and protecting liver cells from toxic effects of more noxious bile acids. It is given by mouth at a dose based on the patient’s weight (13-15mg/kg/day). Probably at least 60-70% of patients get sufficient benefit from UDCA as a sole life-long therapy.
If your blood tests on UDCA don’t approach normal however, then other treatments need consideration. A small number of patients can’t tolerate UDCA.
Obeticholic Acid (OCA) is a newer therapy that is also bile acid derived and works at low doses to improve bile flow, reduce inflammation, and scarring.
Other treatment options include clinical trials of new drugs, or the use of non-licensed drugs such as Fenofibrate or Bezafibrate.
Symptoms of PBC are also treated: there are many options for treating itch, there are strategies for reducing the impact of fatigue, and there are approaches to managing dryness.
What Causes PBC?
The exact cause of PBC is unknown, however there may be a relationship between genetic and environmental factors PBC is a sex specific disease; one in 1000 women over the age of 40 live with PBC, and approximately 90% of patients are women.