Autoimmunity means the body is attacking its healthy cells because it mistakenly detects these cells as harmful to the body. Every part of the body can be affected by autoimmunity, including the liver.
Autoimmune hepatitis (AIH) is the inflammation of the liver that happens when the immune cells attack the healthy liver cells.
Autoimmune hepatitis has been identified in the 1940s but until now, its exact cause is still unknown. It was formerly called chronic active hepatitis. The disease is characterized by liver transaminase increase in the presence of interface hepatitis on histology, autoantibodies, elevated levels of gamma globulin and a great response to corticosteroids.
In fact, the condition happens worldwide but the accurate incidence and prevalence in the United States are unclear. There are many people with the condition but it is unclear or unknown how many exactly. The disease is rare and may become unnoticed unless it’s in the advanced stages already. The advanced stages may lead to complications.
Untreated autoimmune hepatitis may lead to serious complications such liver cirrhosis or scarring and liver failure. However, when the condition is diagnosed early, it can be managed with medicines that can suppress the immune system. In some cases, a liver transplant may become an option, especially in patients who do not respond to medical treatments or when the liver has been severely damaged.
There are two subtypes of autoimmune hepatitis – type 1 and type 2:
Type 1 AIH
Type 1 AIH is considered the most common type in the United States with about 96 percent of the cases. This type is also characterized by a great response to corticosteroids.
In Type 1 AIH, the antibodies present are anti-smooth muscle antibody (ASMA) and antinuclear antibody (ANA). Type 1 AIH may occur along with another autoimmune disease including Crohn’s disease, celiac disease, Graves’ disease, proliferative glomerulonephritis, Hashimoto’s disease, rheumatoid arthritis, Sjogren’s syndrome, primary sclerosing cholangitis, systemic lupus erythematosus and type 1 diabetes.
Type 2 AIH
On the other hand, Type 2 AIH is the subtype that happens in younger patients who are below 14 years old. It happens more severely, it relapses more often and has a worse response to corticosteroids, compared to Type 1. Type 2 AIH happens in an estimated 4 percent of all cases in North America.
In this type of AIH, the antibodies present are the anti-liver cytosol type 1 (anti-LC1) autoantibodies and anti-liver kidney microsomal antibody type 1 (anti-LKM1). People with Type 2 AIH can have the above mentioned autoimmune diseases.
The severity of the symptoms of autoimmune hepatitis depends on the patient’s stage of the liver condition. It is common for some people to experience no symptoms at all. However, as the disease progresses, he or she may feel some things happen in the body including:
- Extreme weakness or fatigue
- Feeling generally unwell
- Joint and muscle pains, which are worse in the mornings
- Abdominal discomfort
- Skin Rashes
- Spider angiomas
- Loss of menstruation (amenorrhea)
- Loss of appetite and weight loss
- Itching or pruritus
- Excessive hair growth in women
- Passing loose stools or diarrhea
In some cases, the symptoms develop quickly over a few days, signaling acute hepatitis. This means that the symptoms develop suddenly or abruptly.
The exact cause of autoimmunity is still unknown. However, many experts say that autoimmune hepatitis happens when the body’s immune system mistakes the liver cells for foreign aggressors. As a result, the immune system makes antibodies to attack the liver cells. The antibodies normally attack bacteria, viruses, and other disease-causing microorganisms.
When the antibodies attack the liver, it can lead to long-term inflammation and eventually, liver damage. Some experts, however, tie the development of autoimmune hepatitis to the gene interactions and some viruses or drugs.
Autoimmune hepatitis happens when the immune system mistakes the liver cells for foreign and harmful aggressors. As a result, the body produces antibodies to attack even the healthy cells. The exact cause of autoimmunity is still unclear but there are risk factors that were identified, including:
Gender – Being female may predispose you to develop autoimmune hepatitis.
Family History – Autoimmune hepatitis tends to occur in families.
Another autoimmune disease – Having another autoimmune disease such as inflammatory bowel syndrome, celiac disease, type 1 diabetes, rheumatoid arthritis, hyperthyroidism, and lupus are more likely to develop AIH.
History of infections – Autoimmune hepatitis may develop after a person got infected with certain illnesses such as measles, Epstein-Barr virus and herpes simplex virus. In some cases, infections of the liver such Hepatitis C, B or A may contribute to the development of autoimmune hepatitis.
Individuals with autoimmune hepatitis are at a higher risk of developing complications from liver cancer. AIH that goes without being treated may cause permanent damage and scarring to the liver. The most common complications of AIH and cirrhosis include:
Ascites – Ascites is the accumulation of fluid in the abdomen. This condition can be uncomfortable and painful, interfering with breathing and other bodily processes.
Esophageal varices – Esophageal varices are the enlarged or ballooning of the veins in the esophagus. This happens when the blood through the portal is occluded, making the blood move into the other blood vessels, particularly in the esophagus and stomach. This is dangerous and may lead to bleeding in the stomach or esophagus.
Liver cancer – Liver cancer happens when tumor cells mutate and spread in the liver. The individuals with liver cirrhosis are more likely to have liver cancer.
Liver failure – Liver failure happens when the liver cells become extremely damaged that the liver does not function properly. When this condition emerges, the only option is to have a liver transplant.
Many people with autoimmune hepatitis have no symptoms and in some cases, the symptoms are vague. Diagnosis is often made when people undergo tests for other conditions and the disease is detected incidentally.
The most common diagnostic tests available include:
Blood tests – Tests are done to determine if there are antibodies present in the body that may signal an autoimmune response like autoimmune hepatitis. These antibody tests can also determine which type of hepatitis a patient has. Liver function tests can also be done through a blood test. This test measures the activity of the enzymes and others that are made in the liver. This test gives an overview whether the liver is damaged or inflamed.
Liver biopsy – The doctors can perform a liver biopsy to diagnose AIH and determine if the liver cells have been damaged. The doctors will insert a needle into the liver to get a liver tissue sample. This will be examined under a microscope.
Ultrasound – The doctor may also request for an ultrasound to check if the liver is inflamed or if scarring is present.
Imaging tests – Imaging tests such as a CT scan or an MRI (magnetic resonance imaging). These tests could produce 3D images of the liver, showing if there are problems such as scarring and lesions, or even cancer.
Treatment is vital in managing autoimmune hepatitis. The treatment is always needed, and early initiation can improve the symptoms. At the same time, early treatment of the disease may help reduce the risk of complications and significantly improve the prognosis of the disease.
The goal of the treatment is to slow or stop the immune system from attacking the liver. This will eventually slow the progression of the inflammation.
Medicines – The most common type of autoimmune hepatitis is prednisone. The other drugs like azathioprine may also be included in the treatment also prednisone.
Prednisone is a steroid medication used as an initial treatment. This drug is used to reduce the inflammation. Initially, the doctor may prescribe a high dose of the drug, which will gradually be reduced over the few weeks.
The drug can also cause many serious side effects when used in the long term such as osteoporosis, bone fractures, high blood pressure, diabetes, glaucoma, cataracts and increased weight.
To prevent the serious side effects of the drug, the doctor may slowly lower and taper the dosage until the lowest possible dose is reached and will be used to control the condition. Moreover, adding the other drug, azathioprine may help prevent the serious effects of steroids.
Early treatment is important. However, there is still no cure for autoimmunity.
The only treatment options available are symptomatic, which means that it just reduces discomfort and manage the various symptoms. In steroids, these are used to reduce inflammation of the liver to prevent further damage and disease progression.
If the liver has been damaged, a liver transplant is the only option. When the liver becomes too damaged that it can’t function anymore, the doctor will prescribe a liver transplant. Though the surgical operation is relatively safe, there are some side effects of this procedure such as rejection of the organ. Thus, a thorough examination of both the donor and the patient should be done.
When the treatment is done early, the prognosis is better. When the condition worsens, it may lead to serious liver damage, failure, and even liver cancer.