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Juvenile Idiopathic Arthritis

Juvenile Idiopathic Arthritis General Information

The juvenile idiopathic arthritis (JIA) is a chronic disease characterized by persistent inflammation of the joints. The JIA is a rare disease that affects about 80 to 90 children out of 100,000. Our immune system protects us against aggressive infections (viruses and bacteria). In doing so, the system may make a distinction between what is foreign and potentially dangerous (that was destroyed) and what is harmless and is considered part of us. It is believed that chronic arthritis is a consequence of an abnormal response (due to unknown causes) of the immune system, as it seems to lose some of its ability to distinguish between foreign cells and our own cells. In patients with this type of disorder, the organism’s immune system attacks the healthy, normal cells that are present in the joints. Disorders that are similar to this disorder (JIA) are called "auto-immune" or "auto-inflammatory" medical conditions, as the immune system reacts against the organs of his own body. However, the precise mechanism that causes AJI (and most other human chronic inflammatory diseases) remains unknown. The Juvenile Idiopathic Arthritis is not a hereditary disease since it can not be transmitted directly from parents to their children. Nevertheless there are some genetic factors, most of which remain to be discovered, that predispose to disease. Even in the event of a genetic predisposition, it is very rare to have two children serving in the same family. There are several different forms of AJI. They are distinguished mainly by the presence or absence of systemic symptoms such as fever, rash, pericarditis (systemic JIA) and the number of joints involved (oligoarticulaire or polyarticular JIA). By convention, the different forms of Juvenile Idiopathic Arthritis are defined according to the symptoms presented for the first 6 months of the disease. For this reason they are also often referred to as a form of beginning.

Juvenile Idiopathic Arthritis Symptoms

"Idiopathic" means that we do not know the cause of the disease and "juvenile" means that the onset of the individual’s symptoms appears before the age of 16. Typical signs of inflammation of the joints are pain, swelling, and limitation of motion. The synovial membrane surrounding the joint, which is usually very thin, becomes much thicker and filled with inflammatory cells, whereas the amount of synovial fluid (called by some "synovium") within the joint, increases. This causes swelling, pain and limitation of movement. A feature of inflammation of the joints is articular rigidity that occurs after prolonged rest, and is therefore particularly pronounced in the morning (morning stiffness). Systemic Juvenile Idiopathic Arthritis it is characterized by the presence, in addition to arthritis, of systemic signs (systemic means that the various organs of the body may be involved). The main symptom of Juvenile Idiopathic Arthritis is represented by a high fever often accompanied by a rash of salmon color that appears during the fever. Other symptoms may include muscle pain, swelling of the liver, spleen or lymph nodes, and inflammation of the membranes surrounding the heart (pericarditis) and the lungs (pleurisy). Arthritis, usually polyarticular (up to 5 or more joints), can be experienced at the beginning of the illness or can appear in the future. The disease can affect children at any age.

Juvenile Idiopathic Arthritis Treatment

When Juvenile Idiopathic Arthritis is to be diagnosed, some laboratory examinations are useful in order to better define the type of AJI and to identify patients at risk of developing severe complications such as chronic iridocyclitis. The goal of the treatment is to enable children to live normally and to prevent or reduce the extent of damage to the joints and organs pending a spontaneous remission of the disease, which occurs in most cases, after a variable time rather predictable. The treatment is mainly based on the use of drugs that inhibit inflammation systemic and / or articular and rehabilitation procedures that preserve the function of the joint and help prevent deformities. The therapy for Juvenile Idiopathic Arthritis is quite complex and requires the cooperation of various specialists (pediatric rheumatologists, orthopedic surgeons, physical therapists, ophthalmologists). The therapy with anti-inflammatory drugs (NSAIDs) is quite common. However, this is merely a symptomatic therapy (this means they it can not cure the disease but it is used to control the symptoms caused by the inflammation). The most widely used anti-inflammatory medicines are the Naproxen and ibuprofen. Aspirin, although effective and cheap, is much less used now mainly for its high toxicity (a therapy with aspirin can trigger toxic effects in the case of high blood levels, liver toxicity particularly in the systemic JIA).

NSAIDs are usually well tolerated; however, gastric disorders are a common side effect among adults, as children are generally not affected by it. The combination of various NSAIDs is not indicated. However, occasionally, an NSAID can be effective where another has not worked. The effect on inflammation of the joints occurs after several weeks of therapy. Articular injections are used when one or a few joints are affected and when the stiffness resulting from the pain becomes annoying. Second level medications are added to previous NSAID treatment for Juvenile Idiopathic Arthritis, which must be continued anyway. It is effective in the majority of patients. The potential toxicity requires monitoring during treatment, with the help of regular blood tests. The combination with folic acid, which is a vitamin, reduces the risk of side effects.

No studies have been conducted properly so far to test the effectiveness on the AJI compared to that of other useful drugs like Cyclosporine or Leflunomide. The Cyclosporine is a major drug for the treatment of macrophage activation that is resistant to steroids. No information is currently available concerning the use of Leflunomide in children. Corticosteroids are the anti-inflammatory drugs that are most effective. However, their use is limited because their long-term therapies are associated with many side effects including osteoporosis and an improper development (failure to grow). However, they are widely prescribed in the treatment of systemic symptoms that have proven resistant to other therapies. The rehabilitation is an essential component of treatment for Juvenile Idiopathic Arthritis. It includes exercises, wearing splints to prevent the continuation of unintended postures. It must be started early and should be carried out routinely to keep the range of motion and muscle strength and prevent, limit or correct deformities.




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