, AIH some nfor to get you started.....

[Guest guest]

http://www.autoimmunehepatitis.co.uk/AIHquestions.htm More info for

you and all. Joanne

Jj Cathcart

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 color is due to the presence of bilirubin, which is the

waste product formed from hemoglobin (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 feces their normal brown color. 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 recognize 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.

jjcathcart@...

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. >>>>>>>>>>>>>>>>>>>>>>>>>

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.