Juvenile idiopathic arthritis is a chronic joint disease that belongs to the rheumatic group. It occurs before the age of 16 and lasts at least six weeks.
What is juvenile idiopathic arthritis?
Juvenile idiopathic arthritis, also known as Still’s disease, is also known by the synonym juvenile rheumatoid arthritis. It is abbreviated JIA and JRA, respectively. It is a chronic inflammatory joint disease called arthritis. The disease belongs to the rheumatic group and occurs in childhood. The disease belongs to the specialty of pediatric rheumatology. Pediatric rheumatism and infantile rheumatism are common collective terms for diseases in the field of pediatric rheumatology. Generally speaking, childhood rheumatic diseases, and thus JIA, belong to the autoimmune diseases. The incidence of JIA is four to five diseases per 100,000 children. Approximately 1000 children develop the disease each year.
Causes
The cause of JIA has not yet been determined. This is also indicated by the “idiopathic” in the name of the disease. The term idiopathic is used for diseases in which no cause can be grasped. Presumably, various autoimmune mechanisms favor the onset of the disease in the presence of a genetic disposition. Infections or trauma may also play a role. In particular, infections with parvovirus B19 are suspected to promote the development of JIA. It is assumed that the body forms antibodies against the virus, which are ultimately directed against the body’s own tissue. This may be due to the fact that the surface structure of the pathogens is similar to the surface structure of certain body tissues. Thus, the antibodies damage not only the viruses but also the body’s own tissues.
Symptoms, complaints, and signs
Different forms of JIA are distinguished depending on symptoms, age, sex, and family history. Systemic juvenile idiopathic arthritis is the most severe form of childhood rheumatoid arthritis. The disease begins with high fever. A small-spotted, salmon-colored rash forms on the trunk, thighs, and upper arms. This may also itch. The children suffer from pain in the cervical spine, which initially occurs mainly when turning the head. After several weeks to months, inflammation of the small and large joints also occurs. In addition to the joints, the lung pleura, pericardium, and peritoneum may also become inflamed. Juvenile polyarthritis with positive rheumatoid factor begins similarly to chronic polyarthritis in adults. Initially, the wrists, finger joints, and toe joints are affected by the inflammations. The inflammations occur symmetrically. Damage, some of it irreversible, can occur within a few months. Rheumatoid nodules may appear on the extensor sides of the arms and legs. The disease may be accompanied by vascular inflammation. The small and medium arteries may be affected. In addition, there are general symptoms such as growth arrest or growth retardation, weight loss, poor performance, or lability. The liver and spleen may be swollen. Juvenile polyarthritis with negative rheumatoid factor begins with subfebrile temperatures, growth retardation, and weight loss over several months. Joints are moderately swollen and slightly hyperthermic. Children are limited in their ability to move. Typically, the finger joints, toe joints, and wrist joints are affected symmetrically. The temporomandibular joints and the joints of the cervical spine may also show functional deficits. In juvenile idiopathic oligoarthritis, the knee joint is most commonly affected. Affected joints are swollen and hyperthermic. Pain occurs only when the joint is moved through its full range of motion. Chronic iridocyclitis develops in 20 percent of patients. Overall, however, the symptoms tend to be nonspecific, making diagnosis difficult. Juvenile psoriatic arthritis is a combination of arthritis and psoriasis. The psoriasis may develop years before the arthritis, or the arthritis may develop a long time before the psoriasis. Typical localizations for psoriasis are the navel region, the extensor sides of the joints and the hairline. The arthritis can affect all joints here.However, initial involvement of the hip joints is typical.
Diagnosis and course of the disease
Fundamental to an adequate diagnosis is a detailed history and a thorough clinical examination. In the blood laboratory, CRP, ESR, rheumatoid factors, ANA, and HLA-B27 are determined. Imaging techniques are used to exclude other diseases. Juvenile idiopathic arthritis must be differentiated from secondary arthritis. During the course of the disease, regular examinations by the ophthalmologist must be performed. Uveitis anterior should be excluded. Uveitis anterior is an inflammation of the iris in the eye. It occurs in 10 to 25 percent of all patients with juvenile idiopathic arthritis.
Complications
Due to this disease, the patient primarily experiences a relatively severe fever. Likewise, a rash may develop on the patient’s skin, which in many cases is also associated with itching. Should the affected person scratch the rash, the itching can usually also intensify. It is not uncommon for inflammation to continue to occur in various parts of the body. This mainly affects the heart and lungs, so that these inflammations can be life-threatening for the patient. Furthermore, the affected person loses weight and also suffers from swelling of the spleen and liver. The patient’s quality of life is significantly reduced and limited by this disease. The joints are also swollen, so that it is not uncommon for the patient to have restricted movement. Children also suffer from growth disorders. The treatment of this disease takes place mainly with the help of medication and can limit the symptoms relatively well. Further complications do not occur. However, if treatment is unsuccessful, surgical intervention may be necessary. Whether there will be a reduction in life expectancy cannot usually be universally predicted.
When should you see a doctor?
Children suffering from a high fever should be examined and given medical attention. Therefore, if the fever persists unabated for several days, a physician should be consulted. If changes in the usual appearance of the skin occur, there is reason for concern. In case of a colored skin rash, the formation of pustules as well as unpleasant itching, a doctor should be consulted. If open wounds form, sterile wound care is necessary. If this cannot be ensured independently, a doctor must be consulted. Otherwise, in severe cases, there is a risk of blood poisoning and thus the premature death of the child. If existing wounds become larger, or if there is pus formation or pain, a visit to the doctor must be made. If there is inflammation of the joints, a reduction in physical performance or restrictions in mobility, a doctor should be consulted. Discomfort of the toes, fingers or hands are signs of inconsistencies that require clarification. If the child shows a listlessness, an increased need for sleep or a withdrawal from everyday activities, a check-up is recommended. In the case of a general feeling of illness, inner weakness, malaise, continuous weight loss or disturbances in growth, comprehensive investigations should be initiated to clarify the cause. Discomfort of the temporomandibular joints and the development of psoriasis are signs of existing diseases that should be presented to a physician.
Treatment and therapy
Nonsteroidal anti-inflammatory drugs are used as the main treatment for JIA. These have an anti-inflammatory and analgesic effect and lower the body temperature. In addition, glucocorticoids are administered intraarticularly or systemically. Because of the sometimes serious side effects, a systemic prescription should be avoided over a longer period of time. If prolonged glucocorticoid administration cannot be avoided, osteoporosis prophylaxis should be given at the same time. If there is no improvement with this therapy, disease modifying antirheumatic drugs such as methotrexate can be used. Methotrexate is also known as MTX. The drug belongs to the group of cytostatic drugs. In addition, biologicals such as adalimumab or etanercept can be administered. However, some drugs from this group are only approved for children over a certain age. In severe cases, pediatric surgical interventions such as synovectomy may be required.
Outlook and prognosis
Juvenile idiopathic arthritis is a type of “pre-existing” arthritic disease of the joints and progresses at a young age just as it would in an older patient. Therefore, the prognosis of a young patient is largely the same as that of an older affected person. There are exceptions when juvenile idiopathic arthritis occurs in early childhood, because then it may have resolved by puberty. This form is more common in girls than in boys. In addition, there are favoring factors in young people, such as a generally better state of health at a young age, an often still higher bone density and resistance, and, depending on lifestyle, stronger supporting muscles around the joints. The young age of the affected patient can thus ensure that joint wear and tear does not progress as quickly as in a patient of the age at which arthritis usually occurs. However, this does not completely halt the progress of the disease. However, the prognosis can be positively influenced by a healthy diet and gentle fitness, which is often more realistically achievable in younger patients than in older people. Because they are usually still healthy and mobile except for the arthritis, they have more opportunities to effectively strengthen the muscles and thus give the joints more stability.
Prevention
Because the causes of idiopathic juvenile arthritis are as yet unknown, there is currently no effective prophylaxis.
Follow-up care
Follow-up care of juvenile idiopathic arthritis is concerned with the correct use of medication on the one hand, and physical and occupational therapy on the other. Exercise therapy aims to relax muscles and relieve pain. Depending on the situation, this may involve passive joint mobilization or supportive treatment. In some cases, the patient’s cooperation is also required to keep the joints mobile and bring about improvement. In order to reduce the medication, ice packs can also be used to relieve the pain. In this case, the patients proceed exactly according to the instructions of the therapist. These measures can easily be carried out at home. There are often appropriate facilities or courses for special water gymnastics suitable for children. In the course of long-term treatment, the affected children and adolescents learn a lot about the appropriate forms of therapy. In addition to their own involvement in physiotherapy, it is also important to use the medication correctly. Here, depending on the age of the patient, the responsibility often lies with the parents in the first instance. Antirheumatic drugs and other agents are mainly used to relieve pain and promote muscle relaxation. Local heat, massage, exercise baths and electrotherapy are also useful methods for muscular relaxation.
Here’s what you can do yourself
In addition to drug therapy, physiotherapy and occupational therapy also play an important role. As part of physiotherapeutic treatment, methods for relaxation and pain relief are used. Passive or assistive mobilization of the joints as well as stretching of affected structures lead to improvement of joint mobility. The targeted activation of the musculature as well as the application of ice packs for pain relief are also part of the physiotherapeutic spectrum. Many of the methods used can also be performed well at home under the guidance of an experienced therapist. Also indicated is a child-friendly type of water gymnastics to strengthen the musculature. Occupational therapy is appropriate for deficits in the hand-finger area. In this case, a focus of this form of therapy is playful, functional training. Individual hand splints are also made as part of this therapy and the use of the splints is practiced. Occupational therapy provides supplementary joint protection training as well as a supply of aids for everyday life for more severely affected children and adolescents. Most of these activities can also be carried out at home with the help of instructions. Social support is helpful for the entire family. The aim is to integrate the chronically ill person into everyday life and into school and training. Information on the use of social assistance and compensation for disadvantages is also included in this support. Self-help groups also make a valuable contribution.
