AutoImmune Hepatitis Support Group | AutoImmune Hepatitis Questions

:: AIH Questions

Click on a question below to see the answer.
1. What does the liver do?
2. What is Auto-Immune Hepatitis exactly?
3. What are the different types of AIH?
4. Who does it effect more, males or females?
5. How much is know of the disease?
6. Is it a common illness or is it a rare, much undiscovered disease?
7. What types of problems are likely to happen with this type of disease?
8. How is it treated?
9. What do the drugs do and how do they help?
10. Will I ever come off the drugs? Why?
11. What can I do, and what can't I do if I have AIH?
12. Will transplantation be needed?
13. What is the survival rate of transplantation?
14. If transplantation is needed, will it come back?
15. Why does it come back?
16. What happens if it does come back?
17. What happens after transplant, what would the next step be?
18. What drugs would be needed to stop rejection?
19. If I have AIH, can I have children?
20. Can AIH be inherited?
21. What is a liver biopsy?
22. What are the dangers of liver biopsy?
23. What is cirrhosis?
24. What are the causes of cirrhosis?
25. Can cirrhosis be cured?
26. Is there any treatment for cirrhosis?
27. Symptoms

1. What does the liver do?
The liver is the body's "engine room". It plays an important role in digestion, it manufactures hundreds of components (e.g. most blood proteins) essential for life, it is a major site of energy production and acts as an energy storehouse, and it assists in removing toxic substances from the blood.
The human liver is comprised of two main segments or lobes: a large right lobe and a smaller left lobe. It nestles against the diaphragm under the rib cage in the upper right part of the abdomen. In adults, it weighs approximately 2-3 lbs (1.0-1.5 kg) and maintains its size in relatively constant proportion to body weight, increasing or decreasing in size as we gain or lose weight. This represents a large excess capacity over what is actually required to sustain life, and we can in fact manage fairly well with only about 20-30% of our livers functioning normally. It is a remarkably robust organ. When damaged, and if the damage can be stopped, or when a part is surgically removed, it is the only organ that has the ability to completely regenerate itself to exactly the right size.
The liver aids digestion by producing bile, a dark orange-brown fluid which is a mixture of cholesterol, various proteins and so-called bile salts - which are powerful detergents. Its colour is due to the presence of bilirubin, which is the waste product formed from haemoglobin (the main oxygen-carrying protein in red blood cells) when old red blood cells are broken down. The bile is secreted via the bile ducts and stored in the gall bladder, from where it is then expelled into the duodenum (the first part of the intestines) when needed. Fatty foods entering the duodenum from the stomach are made more digestible by being emulsified by the bile salts. Bilirubin and its breakdown products are the pigments that give faeces their normal brown colour. It is also the pigment which makes the skin turn yellow in people who are jaundiced. This is because, when the liver is damaged, bile often cannot be secreted properly and the bilirubin tends to accumulate in the blood.

2. What is Auto-Immune Hepatitis exactly?
In a sense, it is a disease in which the body is "rejecting" its own liver. The body's immune system is designed normally to fight infection. When we are infected by, say, a virus, special white blood cells attack the infecting organism and either eliminate it directly or produce proteins known as antibodies that specifically recognise and help to destroy the organism. Quite often, infections are accompanied by some (usually fairly minor) "accidental" damage to healthy tissues, either by the white blood cells themselves or through the production of antibodies (known as auto antibodies) against the bodies own tissues. The same sort of thing can happen when tissues are damaged by chemical substances (such as some types of drugs). In other words, we are all in a state of "autoimmunity", but in most people there is a mechanism which switches off (or controls) autoimmune reactions by our immune systems against our own tissues. In people with AIH, it seems that they are born with (or develop) defects in this control system such that they cannot switch off an autoimmune attack against their own livers. Similar defects seem to be present in people with autoimmune diseases of other organs, such as autoimmune thyroid disease, myasthenia gravis (which affects the nerves and muscles), rheumatoid arthritis (affecting the joints), and some forms of diabetes.
Why are only some tissues affected, e.g. the liver in AIH, and not others? This is because the control mechanism is extremely complex. It seems that it has several components, some that have a general "dampening down" effect on the immune system and others that control reactions separately against each of the different tissues in the body. To develop an autoimmune disease affecting only (or mainly) one organ, it is likely that the general control parts are not working properly and that there are additional defects in one of the parts that control reactions against each tissue separately.

3. What are the different types of autoimmune hepatitis?
Until a few years ago, investigators doing research on AIH classified the disease according to the different types of auto antibodies found in the blood of patients. Patients with antinuclear (ANA) or smooth muscle (SMA) auto antibodies, or both, were said to have Type 1 AIH and those who lack these but have so-called liver-kidney microsomal antibodies (LKM1) were said to have Type 2 disease. About 95% of people with AIH, spanning the whole age range of both males and females, have Type 1. Type 2 patients comprise a small group of (usually) young females with severe disease. Later, it was recognised that some patients have none of these three antibodies but have other auto antibodies that were being discovered. These were classified as having Type 3 AIH and other subdivisions were proposed on the basis of other auto antibodies that were being found. Experience and further research has shown that severity of the disease is related more to the age at which it develops (see question d) rather than to the type of auto antibodies and that, at least from the clinical standpoint, there is little difference between the different types or sub-types. All types respond to standard treatment (see question h) in most cases and there is not much difference in the long-term outcome. Nonetheless, the terms Type 1 and Type 2 are still commonly used because it is felt that the mechanisms of liver damage may be different in these two types - even though the response to treatment and outcome are similar.

4. Who does it affect more, males or females?
Like most autoimmune diseases, it mainly affects females (only about 20% of people with AIH are male). It can develop at any age but the large majority of people with AIH develop the disease between 50 and 70 years of age (often around the menopause in women). It tends to be more severe in younger people. Older people generally have a milder form that is often easier to control with treatment (see How is it treated - question h).

5. How much is known of the disease?
Quite a lot really - at least with respect to its signs and symptoms and how to diagnose and treat it. It is known that people with AIH seem to have a genetic predisposition to the disease (What is AIH exactly? - question b) and that there is probably something, such as a viral infection of the liver (which may go unrecognised), which is required to trigger off the autoimmune reaction in the first place (which probably explains why most people do not have the disease from birth, even though they may be born with the defects). However, the precise nature of the defects, or how to correct them permanently, or how the liver damage is actually caused, is still not known.

6. Is it a common illness or is it a rare, much undiscovered disease?
It does seem to be quite rare, but we really don't know how common it is. Rough estimates suggest that there may be somewhere between 6,000 and 10,000 affected people in the U.K. However, it is now known that many people may have no symptoms for long periods and it is likely that many of those who have milder disease may never be diagnosed as having AIH. In some countries where other diseases of the liver (such as chronic viral hepatitis) are very common, these conditions may mask the AIH and it may go undiscovered.

7. What types of problems are likely to happen with this type of disease?
The large majority of people with AIH respond well to treatment (see question h) and feel pretty well most of the time. The main problem that some people complain of is feeling rather tired from time to time. Also, for reasons that are not understood, in some people the disease progresses to cirrhosis despite apparently adequate control with treatment. Cirrhosis is the term used to describe the deposition of scar tissue in the liver (whatever the cause). This may present its own problems, the main one being an increase in pressure in the blood vessels going to the liver (portal hypertension) which, in turn, may lead to the development of varicose veins (varices) in the stomach and around the lower end of the oesophagus, which may bleed. On the other hand, it is known that people can have cirrhosis for 20 or 30 years without developing such problems, so they may never arise. Other problems that can develop may be due to the drugs used to control the disease (see How is it treated? - question h), but in most cases these are not serious. About 50% of people find that they put on weight when they first start taking the steroids. In about 20% of these, the excessive weight gain causes an increase in blood pressure (which may require treatment). Steroids can also lead to development of diabetes or osteoporosis (thinning of the bones) but, again, at the fairly low doses that are usually required to maintain remission (see How is it treated? - question h), these complications are relatively rare. About 10% of people cannot tolerate azathioprine, either because they develop a rash, or it upsets their stomachs, or it affects their white blood cells. In these cases, slightly higher doses of steroids may be required to maintain remission.

8. How is it treated?
AIH is one of the very few chronic liver diseases that can be very effectively treated by simple drug therapy in the large majority of cases. Corticosteroids (usually prednisone or prednisolone) are the standard treatment. Azathioprine is often used as well, since this has an additive effect which allows for lower doses of steroids to be used, but about 10% of people cannot tolerate azathioprine for various reasons (see What types of problems are likely to happen with this type of disease? - question g). Initially, moderately high doses of the steroids are required for a few weeks or months to get the disease under control quickly. Thereafter, and especially if azathioprine is tolerated, the steroid dose can often be reduced to quite low levels. Three recent studies (in the USA, Sweden and Germany) have indicated that, for most people with AIH whose disease is well controlled, life expectancy is not significantly different from that in the rest of the population. The important thing is to take the tablets exactly as prescribed by the doctor.

9. What do the drugs do and how do they help?
The two main drugs, corticosteroids and azathioprine (see How is it treated? - question h), dampen down the autoimmune reaction. In a sense, they act as "anti-rejection" drugs and indeed they are also used (among other drugs) to prevent rejection after transplantation. Other, newer, "anti-rejection" drugs are showing promise for people who do not respond to this standard treatment.

10. Will I ever come off the drugs? Why?
This will depend partly on how severe your disease was in the first place and how well (and how quickly) it responded to the treatment. As noted in question g, the large majority of people respond fairly quickly and their disease begins to come under control within a few months on treatment, but it seems to take at least one year, sometimes several years, to get it completely under control (i.e. to enter complete remission). Thereafter, it may be possible to stop treatment, but the existing evidence indicates that only about 20-30% of people can remain off the drugs for long periods. There is always the possibility that the disease may return (relapse), even many years after stopping treatment. However, if it does return, it can usually be controlled again by standard treatment.

11. What can I do, and what can't I do if I have AIH?
If your disease is well controlled on treatment, there is really little that you cannot do. However, you should generally avoid alcohol. A glass of wine or half-pint of beer on special occasions will probably do you no harm, but this should really be only "occasional" (e.g. not more than once a month). On the other hand, if your AIH is not completely under control, you can do whatever you feel up to doing but you should avoid alcohol completely. Your doctor is the best person to advise you on this.

12. Will transplantation be needed?
This will depend on whether your disease responds satisfactorily to treatment. As mentioned in questions 8 and 10, the large majority of people with AIH do respond well and therefore never require a transplant. The small number who may need a transplant are those with very severe disease that does not respond quickly enough to drug treatment and the few with long standing disease that has progressed to the point where there is not enough liver left to sustain life or who have developed serious complications.

13. What is the survival rate of transplantation?
Because of improvements in the surgery (and in the drugs used to prevent rejection) over the past 5-10 years, survival rates are usually calculated for only 5 years (because doctors don't yet have enough experience with these new developments over longer periods). Also, because so few people with AIH require liver transplants, experience is still fairly limited. The good news is that, among the centres around the world where the most liver transplants for AIH are performed, 5-year survival is currently about 80-90%.

14. If a transplant is needed, will it come back?
Unfortunately, yes it can. However, the drugs used to prevent rejection can also help to control AIH and it is usually only when these drugs are reduced to low dosages or stopped altogether that the disease recurs. But, if it does come back, it can often be controlled again with standard therapy.

15. Why does it come back?
Probably because transplantation does not cure the basic genetic defects relating to control of the autoimmune reaction (see question b).

16. What are the chances of it coming back?
This is really not known, because the numbers of people with AIH who require transplants is so small and there is not yet enough long-term experience to make these calculations.

17. What happens after transplant, what would the next step be?
This depends on how well the transplant goes. However, most people transplanted for AIH do very well and lead virtually normal lives - even to the extent of sometimes competing in the Transplant Olympics !!!

18. What drugs would be needed to stop rejection?
Previously, the main drugs used were steroids, azathioprine and cyclosporine, in various combinations. However, in recent years tacrolimus is being increasingly used to great effect and other, newer, drugs such as mycophenolate are also giving promising results. (for more information on drugs see drugs section)

19. If I have AIH, can I have children?
If you are a man, there should be no problem. If you are a woman, it will depend on how well your disease is controlled and what (if any) complications you have developed (question i). Your doctor is the best person to advise you on this. If your disease is active, you may find that you cannot become pregnant because active disease can affect ovulation. Recent studies have shown that younger women with AIH whose disease is in remission quite often do become pregnant. Most have no major problems during their pregnancies and have normal healthy babies. However, for reasons that are not understood, some women relapse during their pregnancies and others relapse within a few months after delivery, even if they have continued their normal drug treatment throughout. Therefore, it is important to have very regular check-ups during pregnancy and after delivery, and to recognise that an increase in your medication may be required at some stage. The available evidence suggests that, at the doses normally used to maintain AIH in remission, steroids and azathioprine do not seem to affect the baby.

20. Can AIH be inherited?
Everything about our bodies is controlled by our genes, which we inherit from our parents and their ancestors. But the functions of our genes can be affected by external factors and do change throughout life - which is why we do not enjoy "eternal youth". Furthermore, we all have defects of one kind or another in our genes. Whether these defects affect our lives depends on what they are. In many instances the defects are either not important or there are other genes that can compensate for them. As discussed under Question 2, it seems likely that several defects in control of the immune system are required for AIH to develop, and that these are genetic in nature and are probably inherited. Thus, there seems to be a genetic predisposition to the development of AIH. However, AIH is not hereditary in the usual sense. Rather, it seems that it is more an "accident of nature", in which a number of different genes that predispose to the disease have come together in one individual. There are very few reports of AIH occurring in more than one member of a family, so present knowledge suggests that it is extremely unlikely that it can be passed on to one's children.

21. What is a liver biopsy?
This is a diagnostic procedure used to obtain a small amount of liver tissue, which can be examined under a microscope to help identify the cause or stage of liver disease.

22. What are the dangers of liver biopsy?
Bleeding is the primary risk of a liver biopsy, from the side of needle entry into the liver, although this occurs in less than 1% of patients. Fortunately, the risk of death from a liver biopsy is extremely low, ranging from 0.1% to 0.01%. For more info please visit the tests section.

23. What is cirrhosis?
The liver has a remarkable capability to repair itself when damaged. But when the cause of the damage persists, however, as in chronic hepatitis, the repair process may not be able to keep pace with the continuing cell death. As a consequence, the reticulin framework which holds the hepatocytes in place collapses on itself and eventually begins to stick together, forming scar tissue. This process is known as fibrosis.
If the fibrosis is severe, the scar tissue formed can begin to interfere with the normal passage of blood through the liver. Some liver cells may then become starved of oxygen and nutrients, and this can lead to further cell death and formation of more scar tissue - a sort of self-perpetuating process that can proceed in parallel with whatever is causing the damage in the first place. When fibrosis becomes very severe, it is known as cirrhosis - a term derived from the Greek word kirros, meaning orange or tawny, which aptly describes the appearance of the cirrhotic liver due to the decreased amount of blood flowing through and the accumulation of bile pigments in it.

24. What are the causes of cirrhosis?
It is often thought that cirrhosis is due to excessive alcohol consumption. This is NOT correct. In fact, cirrhosis can be caused by any process that persistently damages the liver. Although heavy drinking of alcohol is the main cause in Europe or North America, this accounts for only about 60% of cases of cirrhosis in these countries. In areas of the world where viral hepatitis and various other microbial infections of the liver are very common, the large majority of cases of cirrhosis are due to chronic infections with these agents. Other important causes include the autoimmune liver diseases and the various genetic disorders that affect the liver.

25. Can cirrhosis be cured?
Once cirrhosis is established the process is essentially not reversible. The only cure is liver transplantation, but this may not always be possible or appropriate. However, the severity can often be reduced by removing or treating the underlying cause of the cirrhosis. For example, individuals with cirrhosis due to excessive alcohol consumption often experience a dramatic improvement when they stop drinking alcohol.

26. Is there any treatment for cirrhosis?
There is no treatment for cirrhosis itself, but the complications that arise in the advanced stages can often be successfully treated, with considerable prolongation of life.
If portal hypertension develops, it is sometimes possible to treat this with drugs to reduce the blood pressure. Other drugs, known as diuretics, are often used to reduce the accumulation of fluid in patients with ascites or other forms of oedema. If these don't work, an operation may be necessary to reduce the ascites or a liver transplant may be required. Gastric or oesophageal varices can be injected with a substance known as a sclerosant to block these off to stop bleeding. This is very similar to the treatment of varicose veins in the legs, except that it has to be done by endoscopy. A more recent development involves using an endoscope to place small rubber bands around the varices to 'choke off' the local blood supply.

If there is severe bleeding, an inflatable balloon (known as a Sengstaken tube) may be passed down the oesophagus to apply pressure as an emergency measure to stop the bleeding until endoscopic injection or the varices can be performed. In some cases, it may be considered necessary to perform an operation, known as a shunt procedure (or TIPS), to divert blood from the portal vein into other vessels that can cope with the pressure.

27. Symptoms
� Fatigue - the most common symptom that people with AIH will encounter.
� Enlarged liver
� Jaundice
� Itching
� Skin rash
� Joint pain
� Abdominal discomfort
� Fluid in the abdomen (ascites)
� Mental confusion
� Amenorrhea

People in advanced stages of the disease are more likely to have symptoms such as fluid in the abdomen (ascites) or mental confusion. Women may stop having menstrual periods. Symptoms of autoimmune hepatitis range from mild to severe. Because severe viral hepatitis or hepatitis caused by a drug--for example, certain antibiotics--has the same symptoms, tests may be needed for an exact diagnosis. Your doctor should also review and rule out all your medicines before diagnosing autoimmune hepatitis.