Story

Attack on your own body

If the immune system gets out of balance, it can turn its weapons against itself. Autoimmune diseases can take a severe course and usually require lifelong treatment. The causes are not yet fully understood.

Text: Helga Kessler

Friend or foe? The human immune system constantly checks whether the material it detects belongs to the body or not. If it finds pathogens such as bacteria or viruses or pathologically altered cancer cells, it deploys its arsenal of weapons. Scavenger cells, antibodies and killer cells ensure that anything identified as “foreign” is removed and the person remains healthy. But as efficient as the immune system is at fighting off pathogens or other foreign material, the consequences are serious if it makes mistakes and turns against its own body. In an autoimmune disease, the immune system attacks the body’s own structures, cells or tissue by forming autoantibodies and activating immune cells. These then attack organs, skin, bones or nerve cells and damage the healthy tissue, causing the affected areas to become inflamed. Once the immune system is set to defend itself against its own material, it can hardly be stopped. Inflammation becomes chronic and causes increasing damage. This can result in progressive and degenerative diseases which, if severe, can lead to death.

Destroyed organs

Which symptoms result and how the disease develops depends on which part of the body is affected. The immune system can specifically target a certain organ and gradually damage or destroy it through chronic inflammation. In Hashimoto’s thyroiditis, the thyroid gland eventually stops producing thyroxine; in type 1 diabetes, the insulin-producing cells of the pancreas fail; in multiple sclerosis, parts of the nerve fibers in the brain and spinal cord are damaged to such an extent that nerve conduction is irreparably impaired; in ulcerative colitis, the mucous membrane in the large intestine becomes inflamed in phases, which can cause severe pain and bleeding. Almost every organ in the body can be affected by an “organ-specific autoimmune disease”.

100 different diseases

If several organs and tissues in the body are the target of the attacks, these are referred to as “systemic autoimmune diseases”. They are particularly complex and can take very different courses in the people affected. In rheumatoid arthritis, it is mainly the joints that become inflamed, in vasculitis the blood vessels and in polymyositis the muscles. Lupus erythematosus is a typical systemic disease in which the skin and often also the kidneys become inflamed in addition to the joints.

Occurrence in the population

Around 100 different autoimmune diseases are known, affecting an estimated five to eight percent of the population worldwide, women four times more frequently than men. Depending on the disease, different ethnic groups may be more severely affected. Some diseases begin in childhood and adolescence, such as type 1 diabetes, others in young adulthood, such as multiple sclerosis or lupus erythematosus. For others, such as rheumatoid arthritis, the risk increases with age.

Rare spontaneous healings

Autoimmune diseases are generally not curable. The disease rarely disappears on its own, as is the case with sarcoidosis, a systemic disease that mainly occurs in the lungs and lymph glands. In seven out of ten people affected, spontaneous healing occurs at an early stage of the disease, and even chronic forms can heal. If, on the other hand, many organs are affected and these are also severely inflamed, the risk of dying from sarcoidosis increases.

Possible treatments

Most autoimmune diseases are treated by inhibiting the inflammation or suppressing the immune system. Depending on the type of disease, either organ specialists or a team of specialists from various clinics will provide treatment. At the USZ, several specialist clinics are usually involved in both diagnosis and treatment. At the invitation of the Department of Immunology, the specialists meet once a week at the interdisciplinary board to discuss complex cases. If a disease is diagnosed in good time, the symptoms can usually be alleviated and consequential damage prevented or at least delayed.

Estrogen as a risk factor

Why the immune system switches to self-attack in those affected is not fully understood. As a rule, several factors have to come together for the disease to break out. Gender, and therefore the different hormonal make-up, plays an important role. A number of autoimmune diseases appear to be promoted by oestrogens. Testosterone on the other hand, the predominant sex hormone in men, appears to dampen the immune system. For example, multiple sclerosis, which affects women three times more often than men, usually only occurs in childbearing age. During pregnancy, MS symptoms usually decrease because the higher progesterone level dampens the immune response. After childbirth, when the hormonal situation changes dramatically again, the symptoms usually return more strongly. Female hormones also appear to play a role in lupus erythematosus – nine out of ten sufferers are women. Here too, the disease usually begins at reproductive age. Men are at increased risk if their testosterone levels are low.

Hereditary susceptibility and harmful substances

However, sex hormones can only partly explain why autoimmune diseases develop – some of them break out before hormone levels change in a gender-typical way. Others, such as ulcerative colitis, affect men and women equally often. Hereditary factors clearly play a role. For example, there are clusters of diseases with colitis, lupus or MS in families. However, it is not the autoimmune disease itself that is inherited, but an increased susceptibility. This could also contribute to the increased incidence of autoimmune diseases in those affected: If MS is present, it is not uncommon for colitis or Hashimoto’s thyroid disease to occur as well. But even identical twins do not necessarily develop the same autoimmune disease. So other factors must play a role. It is known that some environmental pollutants can stimulate the immune system to produce autoantibodies. In the case of sarcoidosis, chemicals or particulate matter absorbed by the lungs are considered possible causes, in the case of lupus UV light. Medication, infections with bacteria or viruses, but also chronic stress and an existing tumor disease can also result in the immunological balance being thrown out of sync. It is also being discussed whether the composition of the intestinal flora makes a difference.