Autoimmune hepatitis (AIH) is an inflammation of the liver caused by your body’s own immune system, causing inflammation in the liver. This disease can be caused by a combination of environmental triggers, autoimmunity (when the immune system mistakenly attacks the body's own tissues or organs) and genetic predisposition. It can present at any age, ranging from children to the elderly. If left untreated, AIH can lead to severe scarring of liver tissue, also known as cirrhosis. Patients with AIH are at risk of developing cirrhosis and liver failure if the condition is not diagnosed and treated early.
Symptoms
Some patients with AIH have no symptoms, although most patients will have one or more symptoms. Common symptoms of autoimmune hepatitis include:
- Abdominal pain
- Fatigue
- Joint pain
- Loss of appetite
- Nausea
- Skin rashes or itchy skin
- Yellowing of skin or eyes (jaundice)
Diagnosis
After a thorough look at your health and family history, and an exam by a physician, blood tests are often done to look at liver function. This helps rule out other conditions and see if there is a presence of autoantibodies (antibodies that mistakenly target and attack a person’s own tissue or organs) that might suggest an autoimmune disorder. Ultimately, a liver biopsy is usually required to confirm the diagnosis of AIH and determine the degree of liver damage.
Treatment
Treatment of AIH should be managed by a hepatologist experienced in treatment of this rare liver disease. Treatment is important in order to suppress the immune system since the immune system is overactive in people with this disease. Early treatment can help to slow progression or even reverse damage to the liver. The most commonly used medications include steroids and other immune suppressing medications. Medications should be carefully adjusted to meet the individual needs of the patient in order to control the inflammation and minimize the risk of side effects from medications. In many cases, the condition can be controlled, although in serious cases, a liver transplant may be needed.
Primary biliary cholangitis (PBC) is a chronic disease of the liver that slowly destroys the small bile ducts within the liver. Bile is made in the liver and aids in digestion as well as helping your body to get rid of cholesterol, toxins and old red blood cells. However, when bile ducts become damaged, bile becomes backed up in the liver and this leads to inflammation of the liver. If left untreated, PBC can lead to severe scarring of liver tissue, also known as cirrhosis. Patients with PBC are at risk of developing cirrhosis and liver failure if the condition is not diagnosed and treated early.
Symptoms
While some people may not show symptoms when diagnosed, it is important to know the early symptoms, which include:
- Dry eyes and mouth
- Fatigue
- Itchy skin
If not caught early, later symptoms can develop including:
- Bone, muscle or joint pain
- Diarrhea
- Fluid buildup in abdomen
- High cholesterol
- Pain in upper right abdomen
- Swelling of spleen
- Underactive thyroid (hypothyroidism)
- Weak and brittle bones
- Weight loss
- Yellowing of eyes and skin
Diagnosis
After a thorough look at your health and family history, and an exam by a physician, the following tests and procedures can be used to diagnose PBC:
- Antibody tests (anti-mitochondrial antibody or AMA)
- Liver blood tests
- Magnetic resonance cholangiopancreatography (MRCP)
- Ultrasound
Occasionally, a biopsy is required although in many patients this is not necessary.
Treatment
Treatment of PBC should be managed by a hepatologist experienced in treatment of this rare liver disease. Medications are available to help slow the progression of PBC, prevent complications and help to reverse some of the injury. Some patients may benefit from clinical trials. If medications are unable to control PBC, or if the condition is detected too late and the liver begins to fail, a liver transplant may be needed.
Primary sclerosing cholangitis (PSC) is a disease of the bile ducts where inflammation causes scars, also known as strictures, within the bile ducts making them hard and narrow. If untreated, the liver can slowly lose functionality and lead to infection of the bile, and severe scarring called cirrhosis. PSC is also highly related to inflammatory bowel disease (IBD), also known as colitis. Patients with PSC are at risk of developing cirrhosis and liver failure, and of developing bile duct cancer (cholangiocarcinoma) and colon cancer. Patients with PSC are also at risk of developing infection of the bile ducts (acute cholangitis).
Symptoms
Some patients with PSC have no symptoms; however, many patients have symptoms, which can include:
- Chills
- Dark colored urine
- Enlarged liver
- Fatigue
- Fever
- Itching
- Night sweats
- Pain in the upper right side of the abdomen
- Weight loss
- Yellow eyes or skin (jaundice)
Diagnosis
After a thorough look at your health and family history, and an exam by a physician, the following tests and procedures can be used to diagnose PSC:
- Liver biopsy in some cases
- Liver blood tests
- MRI of bile ducts (also known as MRCP)
Treatment
Management of PSC aims to manage symptoms or complications and monitor any liver damage. This includes screening for bile duct cancer and colon cancer, treatment of bile duct blockages with ERCP, controlling IBD (colitis), treatment of itching, and nutrition support. It is also important to consider new clinical trials given the absence of effective treatments to date. If PSC causes liver failure, a liver transplant may be needed. We also treat patients with other cholestatic liver diseases caused by elevated serum alkaline phosphatases and by viral, drug or genetic disorders.
