Juvenile rheumatism

Juvenile rheumatism: not only in adults, also in children

Juvenile rheumatism
Juvenile rheumatism is a disease that occurs in one in 1000 children. This condition has the characteristics of chronic or recurring inflammations in the joints. The medical name for childhood rheumatism is Juvenile Idiopathic Arthritis (JIA). This name is not just conceived. These ‘expensive’ words indicate exactly what kind of disorder it is and who is affected by it. ‘Juvenile’ means that the first complaints have arisen before the sixteenth year of life. “Idiopathic” means that it is not known what causes the disorder. And with ‘arthritis’ it is made clear that this is an inflammation of the joints. Juvenile rheumatism is not a single condition, but a collective term for various forms of joint inflammation in children. The most important are: oligo-articular juvenile rheumatism, poly-articular juvenile rheumatism and systemic juvenile rheumatism. The ultimate form of childhood rheumatism can only be established half a year after the first symptoms occur.

 

Oligo-articular juvenile rheumatism

This form is most common in children. In the case of oligo-articular, a maximum of four joints are simultaneously inflamed. This condition mainly affects the large joints – often left and right at the same time – of the body, such as knee, ankle or elbow. The inflammations occur at the spot where the tendon is attached to the bone. If the first complaints for the sixth year of life occur, we will talk about early-onset oligo-articular juvenile rheumatism. This form mainly occurs in girls. In this form, anti-nuclear antibodies (ANA) can be present in the blood. This increases the risk of eye inflammation. In boys, the joint inflammation in many cases only manifests itself in puberty. Then we talk about late-onset oligo-articular juvenile rheumatism.
How this form of youth rheumatism will develop cannot be predicted. In most cases, the joints become inflamed a few times. After that, the complaints do not return, without the joints getting damaged. In some cases, the joint inflammation can expand further in the body and develop into chronic rheumatism. This can lead to rheumatoid arthritis, psoriatic arthritis (rheumatism with psoriasis) or stiffening of the back. The latter condition in adults is called Bechterew’s Disease.

Poly-articular youth rheumatism

In this form, five or more joints in the body are inflamed. The inflammations are present in the small joints of the hands and feet. The rheumatic factor (RF) is present in the blood in less than 5 percent of children with childhood rheumatism. Then the symptoms of the polyarticular juvenile rheumatism strongly resemble those of rheumatoid arthritis in adults. The inflammation can then spread to the large joints of the body. The symptoms start around the age of 10 and can be long lasting and severe. This can eventually lead to permanent damage to the joints.
Even if the rheumatic factor is not found in the blood, little can be said about the course of the disease. In some cases the inflammation decreases rapidly, in other cases it can take a while before the disease has regressed. In most cases, the joints remain undamaged enabling them to function properly over time.

Systemic youth rheumatism

In this type of youth rheumatism not only the joints are inflamed, but also different organs. The most important symptoms of systemic juvenile rheumatism are, in addition to pain and stiffness in the joints, high fever and skin rash. In addition, the liver, spleen and lymph nodes can be enlarged. The child will usually not notice a lot of inflammation of these organs. This is indeed the case with inflammation of the pericardium or pleura causing pain in the chest.
A serious risk of macrophage activation syndrome (MAS) is present in systematic juvenile rheumatism. A macrophage is also called the large ‘eating cell’. They crawl through the tissues and absorb viruses and bacteria. In MAS the macrophages are active beyond control. In the bone marrow they will eat the new blood cells, causing a shortage of red and white blood cells and platelets. This leads to high fever, anemia, problems with blood clotting and increased susceptibility to infections. Systemic childhood rheumatism is a persistent condition that is difficult to treat.

What is the cause of childhood rheumatism?

That is still a big mystery. However, it has been established that it is an autoimmune disease. In short, this is a disruption of the body’s immune system. This immune system has the task of detecting and defusing everything that does not belong in the body such as harmful viruses and bacteria preventing these malicious invaders to make us sick. In an autoimmune disease, the immune cells receive a wrong signal for a still unknown reason. They also deal with the healthy cells of the body. This causes inflammation in the joints and sometimes also in the organs.

How is youth rheumatism treated?

To date, no specific drugs for childhood rheumatism have yet been developed. All drugs for rheumatism are exclusively tested on adults. It is still insufficiently known how they work in children. Worldwide (children’s) rheumatologists regularly discuss the effects and side effects of such drugs in children.
In the case of mild joint problems, the doctor will prescribe a simple painkiller such as paracetamol. The child then has less pain and can therefore move more easily. If this does not work, you can switch to NSAIDs. However, these anti-inflammatory painkillers only have effect for a few hours. The child will therefore have to take several tablets per day. The use of NSAIDs is associated with side effects, such as stomach problems or bruising more quickly. Corticosteroids inhibit the inflammation and limit the damage to the joints.
Prednisone is the best known agent in this respect. This drug can also be given as a local injection into the joint. This has the advantage that the drug only works where the inflammations are present. However, this method of treatment (via an injection) has proven to have a long-term harmful effect on the cartilage in the joints.
When these drugs do not give enough results, the doctor may prescribe a classic rheumatic inhibitor (DMARD). These drugs make the disease less active, which means that less damage to the joints will occur. It can take weeks to months for the effect of a DMARD to become evident. If this is not the case, the doctor may prescribe a biological rheumatic inhibitor. This contains animal or human protein that can stop inflammatory processes. This is given by injection or infusion.

Is childhood rheumatism hereditary?

The three main forms of childhood rheumatism are not hereditary. It occurs very sporadically that several children in a family suffer from this condition. Genetic predisposition does play a role in the development of childhood rheumatism. Little is known about this, but a lot of research is being performed on this subject. Some forms of youth rheumatism can be hereditary. This applies to enthesitis related arthritis and psoriatic arthritis. These forms of youth rheumatism can more often occur within one family. In the first type of juvenile rheumatism, the tendon attachments to the bone are inflamed next to the large joints of the lower part of the body. The hereditary factor HLA-B27 is detectable in the blood in this condition. In psoriatic arthritis, joint inflammation is associated with the skin disease psoriasis. Both of these forms of childhood rheumatism are therefore inherited.

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