LIVER CONDITIONS
Auto Immune Conditions
Auto-immune means that the body’s natural defence mechanisms, known as the immune and inflammatory systems, which in normal conditions are vital for defending and repairing the body against injury and illness, is for some reason (usually genetic or viral induced) working incorrectly. The system consists broadly speaking of two main components. Firstly a reaction that is triggered by the body to a foreign substance for instance direct injury or infection by a virus or bacteria. This causes an increase in certain cell production and blood flow to the area of injury or invasion, which is called the inflammatory response, and works by saturating the area with cells to fight invasion. The immune response works in conjunction with the inflammatory response and specifically deals with recognising foreign cells and blocking their ability to reproduce and survive. We build up natural immunity over many years as our bodies immune systems memorise and therefore respond more efficiently to repeated infection. We now recognise that in some individuals this system becomes faulty and this can be selective causing specific organs of the body to be targeted or it can be systemic where the whole body is attacked. Diseases of the liver that are referred to as ‘autoimmune’ are specifically Primary Biliary Cirrhosis (PBC), Primary Sclerosing Cholangitis (PSC) and Auto immune hepatitis (AIH).
PBC is a disease of the liver where the bile duct system is predominantly affected by the faulty immune function. Over a period of time the inflammation in the bile ducts impairs the flow of bile and this affects the liver tissue causing inflammation and with time jaundice and cirrhosis. A lot about PBC is not truly understood and at present the disease seems to be genetically linked, mainly occurring in females, often lying dormant until patients reach middle age. The symptoms of PBC relate to the fact that it is what we call cholestatic in nature (impairs bile flow) and therefore jaundice and itching are the main side effects. As with all liver diseases, as the degree of cirrhosis increases then the symptoms will progress and the aim of treatment is to reduce and monitor this occurrence. Ultimately PBC may take many years to present a truly cirrhotic picture and patients who are well should be monitored on a six monthly to yearly basis dependent upon age and symptoms. Patients must be under the care of a Hepatologist and the long term use of drug therapies is discussed on an individual basis. Transplantation is a valid treatment for those with PBC where the symptoms related to the disease impact upon the quality of life.
PSC is a disease that is seen predominantly in men and is strongly linked to inflammatory conditions of the bowel namely ulcerative colitis. Its cause is unknown and there may be a genetic link or a viral link but this has not yet been proven. PSC is characterised by narrowing and twisting of the bile ducts both inside and outside of the liver and this leads to disruption of bile flow causing inflammation and infection. The infection leads to increased narrowing with scar tissue formation and the process is progressive. Drug treatments are required and long term the aim is to stop inflammation and guard against infection. Monitoring with regular blood tests and pictures of the biliary tract are essential and if stable then this is usually done at six month intervals but this is very dependent upon what degree of bile duct damage is present and the interval between bouts of infection. Most patients manage their PSC at home with blood tests and drug dosages discussed on an out patient basis. Ultimately liver transplantation is considered for those who suffer repeated patterns of infections and jaundice and when the symptoms of decompensated liver disease become apparent.
AIH is a condition where the liver cells are targeted by the immune system and it is most commonly seen in women. With some patients there is a pre-existing auto immune condition like rheumatoid arthritis or thyroid disease evident. Common symptoms of AIH are itching and lethargy and although it is mainly a chronic disease of the liver it can be acute. AIH differs from other auto-immune conditions in that it is very responsive to drug therapy and with correct medication life expectancy can be normal.