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With years of media attention and scores of high-profile cases, most people are at least somewhat familiar with liver diseases such as hepatitis A, B and C and the causes associated with them. Tainted blood, intravenous drug use, unprotected sex with multiple partners and unsanitary conditions in food handling all figure into the mix of viral hepatitis. But what do these viruses have in common? The answer is that they are generally associated with some sort of risk factor on the part of the patient. Regardless of how innocent, the risk involves some action by the hepatitis patient, even if that action is inescapable, as in the case of a life-saving blood transfusion. Autoimmune hepatitis (AIH), on the other hand, is different. It is a disease in which the body’s immune system, rather than a virus, attacks the liver, causing it to become inflamed. Researchers suspect that a genetic factor might predispose some people to autoimmune diseases and that something in their body might trigger the autoimmune response, so the disease manifests itself without risk factors. Like viral forms of hepatitis such as HBV or HCV, the disease is usually quite serious and, if not treated, gets worse over time. It is usually chronic – meaning it can last for years – and can lead to cirrhosis and, eventually, liver failure. According to the National Digestive Disease Information Clearinghouse, AIH is classified either as type I or type II, with type I being the most common form in North America. It occurs at any age and is more common among women than men. About half of those with type I have other autoimmune disorders, such as type 1 diabetes, proliferative glomerulonephritis, thyroiditis, Graves’ disease, Sjögren’s syndrome, autoimmune anemia and ulcerative colitis. Most AIH patients – about 70 percent – are women between the ages of 15 and 40. Type II autoimmune hepatitis is less common, typically affecting girls ages 2 to 14, although adults can have it too.
Living with AIH
Devanee Auel of Elkhorn, Neb., has lived with AIH for about five years. She says she feels pretty good these days, but getting to this point was the tricky part. In December 2000, Auel recalls, she applied for a life insurance policy that required her to give a blood sample. When the results came back, it showed she had mildly elevated liver enzymes.“I went to my family doctor and he ordered the hepatitis viruses to be checked. They came back negative and he then ordered the rare liver diseases tests and sent me for an ultrasound to check for a fatty liver,” she says, recalling that those tests all came back normal as well. “We waited a few months and checked for the hepatitis viruses again because he said it may take time for the antibodies to show up. (But) they were negative again.” She says she was referred to a gastroenterologist who told her some people naturally have slightly elevated enzymes and that she should have them checked annually, but that she probably need not worry.Though the “diagnosis” never set well with her, she continued to have her levels monitored for a couple of years. After moving to another city, she says she provided her medical history to her new family doctor, who referred her to another gastroenterologist in October 2003.“She (the new doctor) was angry at what the other GI doc told me and said something was causing my enzymes to be high and we needed to find out what,” she says. “She redid all the liver disease tests and ultrasound and they came back normal.” It was still more than a year, however, before Auel finally was diagnosed with AIH, and then only after other forms of hepatitis, such as HCV, had been ruled out. But once properly diagnosed, she was able to get the disease under control with medication. “My (liver functions) have been normal for nine months and I feel fine,” she says. “The only inconvenience with the disease now is popping a pill every morning.”Dr. Howard J. Worman, an associate professor of medicine, anatomy and cellular biology at Columbia University’s College of Physicians and Surgeons in New York City, says AIH can be a tricky diagnosis. “Most liver specialists can diagnose it,” he says. “However, among primary care physicians, it is frequently misdiagnosed.” Dr. Bennet Cecil of the Hepatitis Treatment Center in Louisville, Ky., says that while AIH is considerably less prevalent than other forms of hepatitis, it is still difficult to get a grasp on exactly how many people are affected by it. “It’s hard to know how many people are out there with autoimmune hepatitis that go undiagnosed,” he says. “We don’t think of it as being silent, but I don’t know of any study where they’ve actually gone out and taken a bunch of people and screened them for autoimmune hepatitis. Autoimmune hepatitis is much less common than viral hepatitis.”
Drug therapy
People with autoimmune hepatitis commonly are treated with corticosteroids such as prednisone, or prednisolone, the form it must take to be active. The drug is effective, but patients can experience some side effects, including hypertension, sodium and water retention, potassium loss, mental disturbances, euphoria, muscle wasting, and possibly peptic ulceration. Corticosteroids also might cause Cushing’s syndrome, suppressed growth in children, adrenal atrophy, and, if administered during pregnancy, might affect adrenal gland development in the child. Suppression of the symptoms of infection also might occur. Another commonly used drug, azathioprine, or Imuran, can be associated with hypersensitivity reactions including dizziness, malaise, vomiting, fever, muscular pains and shivering, joint pain, jaundice, heart arrhythmias, low blood pressure (requiring withdrawal of treatment), symptoms of bone marrow suppression, hair loss, increased susceptibility to infections, nausea, pneumonia, and pancreatitis. While it is common for most people to experience at least some side effects, the drug therapy keeps liver damage in check and enables most patients to live without symptoms.“Most patients respond to treatment with a decrease in liver inflammation and symptoms, if they had any, when given prednisone – or prednisolone – with or without azathioprine,” Dr. Worman says. “In some patients, you can taper the drugs and stop treatment or significantly decrease the dose. Some patients need low doses of these drugs for a long time to control the hepatitis.” “It works well in most people. Not everybody, unfortunately, but most people,” Dr. Cecil adds. “We use immunosuppressant drugs for them and it is frequently successful.” Dr. Cecil says one patient, whom he has been treating for about 15 years with immunosuppressant drugs, has responded to the point that his cirrhotic liver has healed itself to some degree.“Now, if I withdrew the immunosuppressant drugs, he may still do fine or he may reactivate again. We discussed that and, for this particular man, we’re just leaving well enough alone. Oftentimes, what we do is to continue the treatment for many, many years and people generally do very well,” he says. “I’ve got another lady, though, who, despite her treatment, has progressed and needs a liver transplant. So, in spite of trying different drugs and trying high doses, it didn’t work for her. Her own body destroyed her liver and now she has to have a new liver, so there is a spectrum.” Dr. Worman says that getting the disease in check does not necessarily mean patients are out of the woods. “The problem is that despite appropriate treatment and an improvement in liver inflammation, a significant number of patients, perhaps 50 percent, develop cirrhosis over a 10-year or longer period,” he says. “If complications of cirrhosis develop that indicate liver transplantation, transplantation is usually effective.”
Getting the word out
Matt Hastings learned about his condition much like most others with AIH: a trip to the family doctor after falling ill for unexplained reasons. Like most, he was referred to a specialist where he was put through a battery of tests before ultimately being diagnosed. But the 25-year-old Great Yarmouth, England, resident’s similarities with most AIH patients end there. Hastings stands out on a number of points. First, Hastings is the recipient of two liver transplants. The first one was in July 1999, just four years after being diagnosed at the age of 14. The second was performed in February 2005. But perhaps it is what he has done with the disease – rather than what AIH has done to him – that sets him apart most from others. In the year following his first transplant, at only 18, Hastings launched a website devoted not only to being a clearinghouse of information about the rare disease but also a support mechanism for those with AIH. “I’ve provided a forum which is a place for people to chat or have a moan about the illness, give advice and support fellow sufferers,” Hastings says. “Most of the website runs itself; it just needs updating every few months, and I need to reply to e-mails.” Hastings has loaded the website, www.autoimmunehepatitis.co.uk, with information about the disease and with links to other websites around the world. Though the bulk of the information has a distinct British slant, Hastings says plans are to include considerably more information relevant to U.S. and North American subscribers.“At first, I didn’t think it would be popular, but how wrong was I. It was an idea that blew up in my face,” he says. “It was just what people needed – a place to come and chat and get information in plain understandable English so that a child could understand it. It now has 300-plus members on the mailing list from around the world.” Margaret Saldana of Austin, Texas, is one of those members. Diagnosed in 2002, Saldana says she is glad Hastings has provided a place for her and others to share their stories of hope and recovery.“The autoimmune hepatitis site has helped me a lot,” she says. “At first, I found some other sites, but they depressed me, so I stopped. About a year ago, I found AH and I was glad I did. I have not met anyone else in Austin that has autoimmune hepatitis. I know several people that have hepatitis A and hepatitis C. So I tell people about my illness. I have not heard if there are any support groups in town, so AH has been my support.”
The Least Among Us
When Sabrina Williams was 3 years old, she had a bout with an unhealthy looking grayish stool. Her mother, Susan Williams, didn’t get too concerned because unpleasant stools are not uncommon with toddlers. “With kids, sometimes it turns a funny color when they are getting sick, so we didn’t think anything of it,” she says. That was soon to change. “She got up one morning and her eyes were completely yellow,” Williams recalls. “I took her in and they drew blood and then they called me later that night and said she’s got some form of hepatitis, so they got us in the next day and she was actually put in the hospital.” Williams says her daughter’s liver functions were so off the chart that doctors immediately started treating her with steroids. That course of action proved to be the correct one and her numbers quickly normalized. “Luckily, I took her in because they said she probably wouldn’t have made it another week, which is bizarre because other than the gray stool and her eyes turning yellow, she never had any other symptoms,” Williams says. Doctors diagnosed Sabrina, now 7, with autoimmune hepatitis (AIH), a disease in which the body’s immune system “rejects” its own liver. Left untreated, the disease can cause severe liver damage. “The way they explained it to us is every organ in your body has, say, a serial number that is stored up in the brain,” Williams says. “Your body is constantly checking to make sure all the serial numbers are correct. Well, with her liver, the serial number is not the same, so her body is saying, ‘Attack it. Kill it.’ So, basically, her body is trying to destroy her liver.” To control the disease, Sabrina is forced to take cyclosporine, a medicine normally given to transplant patients to prevent their bodies’ rejecting a new organ. After she was diagnosed, Sabrina had a biopsy that showed about 10 percent liver damage. “Two years after that she had another one and amazingly most of the damage was gone,” Williams says. “The liver can regenerate, but with her body constantly trying to kill it, it’s kind of unheard of.” Williams says her daughter otherwise is healthy and normal. She must continue taking the medication to control the disease, but it has its drawbacks as well, causing excessive hair growth and Sabrina’s gums to swell – an unfortunate trade-off in the battle to save a little girl’s life.
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