Autoimmune hepatitis is a chronic disease that occurs when the body’s immune system which is set up to protect the body from invading bacteria, fungi, and viruses attacks its own cells and body systems. In this case, the immune system attacks the cells of the liver, causing inflammation, and can ultimately lead to cirrhosis and liver failure if left untreated.
Definition & Facts
There are two classifications of the disease. Type 1, also known as classic autoimmune hepatitis, is the more common manifestation of the disease, affecting women between the ages of 15-40. Type 2 is rarer, usually affecting young women between ages 2-14. A large majority of all patients who suffer from the disorder are women, and people from all ethnic backgrounds are equally at risk. Comorbid conditions include other autoimmune diseases including Type 1 diabetes, digestive system disorders like ulcerative colitis, celiac disease, and Crohn’s disease, and vitiligo.
Symptoms & Complaints
If left untreated the disease can cause cirrhosis, or severe liver damage; ascites, which is an enlarged abdomen due to fluid shifting to extracellular spaces and accumulating; severe mental confusion from ammonia building up the body; and eventually in liver failure. Approximately 15% of cases may present as a severe case of acute hepatitis, but in most cases the onset is sudden and patients present with flu-like symptoms.
There is no specific scientific attribute that causes a person to develop autoimmune hepatitis. All autoimmune diseases are characterized by the immune system attacking body cells as if it were defending the body against invading pathogens. Factors including heredity and prior viral illnesses may play a contributing factor towards a person develops an autoimmune disease and having a preexisting autoimmune disorder also increases the risk of developing the disorder. There is ongoing research to pinpoint the specific cause of this group of diseases in the hope that prevention and treatment will be more streamlined for patients.
Diagnosis & Tests
Autoimmune hepatitis is diagnosed after a thorough assessment is conducted by a healthcare provider, and by laboratory tests including blood panels and a liver biopsy. A complete blood panel for an autoimmune disease tests for specific antibodies which helps to distinguish the autoimmune disorder from other liver disorders that have similar clinical manifestations. Blood will be drawn at the clinic or healthcare provider’s facility and is used to test, count, and measure the pathogens and blood components circulating in the patient’s body.
A liver biopsy is a scheduled outpatient procedure, which means that the patient does not stay in the hospital afterwards, and involves planning and patient education prior to performing. The patient is usually medically sedated but not fully put under anesthesia; another medication or local anesthesia is used to control the pain.
A hollow needle is inserted and guided by ultrasound or computerized tomography (CT) and used to extract a small sample of tissue from the liver. The sample is put on a slide and examined under a microscope to determine the type and extent of liver inflammation and cellular damage the patient is experiencing. The patient will need to be still throughout the procedure and the immediate recovery period is about two hours.
There is some risk of infection when the skin is punctured, and the patient should arrange for someone to drive them home after the procedure, as they will still have sedative medication in their system. The patient may need to modify their medication regimen prior to the procedure, as certain medications may interfere with dyes or injected substances. It is best to thoroughly discuss and develop a plan with the health care provider who will be performing the procedure to ensure that all instructions are understood and adhered to.
Treatment & Therapy
The treatment approach towards autoimmune hepatitis is not to cure it, but to suppress inflammation so it does not cause further hepatic damage. It is most often treated with a corticosteroid called prednisone, a drug that works by suppressing inflammation and swelling, hampering activity of the immune system. Side effects of corticosteroids include weight gain, osteoporosis (weakening of bone tissue), elevated blood sugar, elevated blood pressure, vision changes related to elevated pressure in the eyes like glaucoma and cataracts, hair loss, and emotional changes including anxiety.
Prednisone is started at a high dose to ensure maximum effectiveness and then tapered to the lowest dose possible. It is important to never abruptly stop treatment with prednisone, as there can be serious adverse effects. Health care providers may augment the lowered dose of prednisone with a second drug, called azathioprine, which acts as an immunosuppressant. Side effects of azathioprine may include nausea, vomiting, and skin rash.
It is important to contact a healthcare provider if a low grade fevers occurs (over 100.4 degrees Fahrenheit) in order to seek antibiotic treatment, as the body is not able to fight off infections effectively while on immunosuppressant therapy, especially if the two medications are combined. It is also very important to obtain periodic white blood cell counts when receiving immunosuppressive drugs to ensure that the count is not low enough to cause bone marrow suppression, which can lead to severe susceptibility to diseases.
Prevention & Prophylaxis