Still Syndrome

Still Syndrome

Still ‘s syndrome is a form of rheumatism that occurs in children. The disease affects the entire body, causing fever, rash, and swelling of the lymph nodes, liver, and/or spleen. Three quarters of those affected are symptom-free as adults.

What is Still Syndrome?

Still ‘s syndrome is a rheumatic disease of children and adolescents under 16 years of age. Boys and girls are affected about equally often. It is also known in medicine as systemic juvenile idiopathic arthritis (sJIA). The addition “systemic” indicates that the disease is not limited to individual parts of the body, but extends to the entire organism. For what is hypoparathyroidism used for, please visit fun-wiki.com.

When Frederic Still first described the syndrome named after him, the causes were still completely unclear (“idiopathic”) – even today there are only rough theories about its origin. In addition to Still’s syndrome, there are other variants of JIA; About 10 to 20 percent of all sufferers suffer from the systemic form.

Causes

Although various theories exist about the causes of Still’s syndrome, there is still a great need for research to understand the mechanisms behind the development of the disease. Researchers are discussing both genes and environmental influences. A theory of the origin of Still’s syndrome deals with the immune system.

Certain genes may trigger an autoimmune reaction, in which the body’s defense system mistakenly turns against itself. This is indicated, among other things, by some symptoms of Still’s syndrome; for example, it is typical of autoimmune diseases that the lymph nodes swell or a skin rash develops.

In addition, however, medicine also considers external influences as a cause. For example, she discusses the extent to which an infection is a possible trigger. Parvovirus B19 seems to show great promise in this regard. It primarily affects people and, after successful infection, causes ringworm. In this disease, the skin rash (exanthema) spreads over the face and trunk.

On the arms and legs, it only manifests itself on the extensor side. Fever and itching are other symptoms. Ringworm is one of the rare diseases today and usually does not have any serious consequences or complications. However, infection with ringworm increases the incidence of arthritis – the possible link between human parvovirus B19 and Still’s disease.

Symptoms, Ailments & Signs

The affected child initially has a fever every day, after which at least one joint in the body becomes inflamed. The fever lasts two weeks or more, possibly flaring up and down, but recurring every day. The joint inflammation shows up within the next six months. A salmon-colored rash that is fickle and patchy may spread across the skin.

Still’s syndrome also has a very strong effect on the internal organs. In addition to the lymph nodes, the spleen and liver may also swell. The thin skin surrounding the organs (serositis), the heart ( carditis ), the iris and/or the ciliary body ( iridocyclitis ) may become inflamed. In addition, those affected may suffer from anemia.

Diagnosis & course of disease

Still’s syndrome usually begins insidiously between the ages of two and eight, often before the age of four. After the first fever, the joint inflammation manifests itself over a period of six months. The finger joints often become inflamed first; Medicine speaks of the “still hand” when the joints swell symmetrically in a spindle shape.

The disease is usually chronic; Three quarters of the patients no longer suffer from the symptoms in adulthood. In the worst case, however, severe damage to the joints is the result. A detailed anamnesis is of great importance for the diagnosis. Doctors examine the blood and can thus detect signs of inflammation. X-rays of potentially affected joints not only show how widespread the joint inflammation is throughout the body, but also how severe the destruction of the individual joints is.

Complications

Still’s syndrome causes many different symptoms. As a rule, the affected children suffer from a very high fever. There is fatigue and exhaustion. Inflammation also occurs, which in most cases affects a joint. Several joints can also become inflamed. The children suffer from severe pain and swelling.

Due to the pain, there are also limitations in movement and thus also in development. Discomfort also occurs on the skin, so that it is reddish in color and may also be covered with a rash. If Still’s syndrome is not treated, the spleen and liver will also be irreversibly damaged. The child can eventually die from this damage.

The heart can also become inflamed and those affected often suffer from anemia. There are no special complications in the treatment of Still’s syndrome. Inflammation can be treated with medication. It also relieves pain and swelling. However, various therapies are still necessary to restore full movement of the affected joints.

When should you go to the doctor?

If children show health impairments or abnormalities, they should always be presented to a pediatrician. During a control visit, the severity or extent of the peculiarities can be discussed and clarified. Swelling of the lymph, fever or changes in the complexion should be examined and clarified. If deformations or irregularities are noticed on the upper body, a doctor is also needed. Inflammation or limitations in joint activity, locomotion disorders and a decrease in physical performance must be presented to a doctor.

If the child shows general behavioral problems, tearfulness or an apparently unfounded aggressive appearance, it needs help. In many cases, physical irregularities are behind this occurrence. Listlessness, apathy or irritability are further signs of an existing health disorder. If the child’s play instinct is reduced, sleep disorders or withdrawal behavior appear, a doctor should be consulted.

Spots on the skin that spread or develop on other parts of the body are considered uncommon. Particularly warm joints or a feeling of heat in the body are further indications of an existing disease. Swelling in the hands or feet is uncommon and should be investigated further. If the child has a fever for several days, there is a need for action. Although the increased body temperature regulates itself over the course of the day, repeated occurrences are a sign of illness.

Treatment & Therapy

When treating Still’s syndrome, doctors usually prescribe anti-inflammatory painkillers, so-called nonsteroidal anti- inflammatory drugs (NSAIDs). They relieve the symptoms and should stop the joint destruction. In contrast to the previously widespread steroidal anti-inflammatory drugs, NSAIDs have fewer side effects. Medicine has been using NSAIDs since the 1950s.

Non-selective NSAIDs or COX-1/2 inhibitors include aspirin, ibuprofen, and indomethacin. Cyclooxygenases (COX) are enzymes that play a central role in the inflammatory response. Drugs that inhibit the biocatalysts accordingly interrupt the inflammatory reaction or at least dampen it. Cyclooxygenases occur in two forms in the human body; however, only COX-2 is critical for the treatment of Still’s syndrome and other rheumatic diseases.

If an NSAID inhibits both cyclooxygenases, more side effects occur. In a specific individual case, however, a non-selective NSAID may make more sense; Only the attending physician can decide which drug offers the greatest benefits. Drugs that only inhibit COX-2 have been on the market since 1999. Examples of selective NSAIDs or COX-2 inhibitors are rofecoxib, parecoxib, etoricoxib and celecoxib.

In addition to NSAIDs, other drugs can also be considered, including glucocorticoids and, from a drug-specific age, also biologicals. Occupational therapy and physiotherapy complement the treatment. Psychological or psychosocial support should also be provided for children who are severely affected by the consequences of Still syndrome or who show behavioral problems, unusual anxiety or depressive symptoms. In the case of severe and/or systemic diseases such as Still’s syndrome, those affected are often also psychologically stressed.

Prevention

Since the exact causes of Still’s syndrome are still unclear, targeted prevention is currently not possible. If the theory that the human parvovirus B19 causes the disease is confirmed, vaccinations could bring progress.

Aftercare

Still’s syndrome requires extensive follow-up care. Patients suffering from Still’s syndrome should contact their general practitioner. The relapsing course of the disease must be monitored by a doctor. If you have a high fever and severe joint pain, the doctor may prescribe a painkiller or other medication. After recovery, the doctor informs about the correct tapering of the drug.

Depending on which preparation was prescribed, any side effects must also be clarified. In the case of chronic symptoms, Still’s disease must be examined regularly by a doctor, since the disease poses a continuing risk. Comprehensive medical treatment is necessary, especially for young children.

Follow-up care for Still’s disease is carried out by the family doctor or the responsible specialist who was already involved in the treatment of the syndrome. This is usually an internist or an orthopaedist. Other contacts are general practitioners or physiotherapists if the joint pain persists after recovery.

Still’s syndrome occurs primarily between the ages of one and four. If you have a predisposition, you should consult a doctor during this period so that the disease can be clarified or ruled out. The pediatrician is usually also involved in the follow-up care.

You can do that yourself

To support the recovery process, relatives should create optimal conditions for strengthening the immune system for their offspring. The disease mainly affects children and adolescents. Since they are naturally unable to initiate sufficient self-help measures, they are dependent on the help of relatives and adults.

They are obliged to inform the sick child of the health disorder and the symptoms. A comprehensive explanation of the further course and the avoidance of risk factors is necessary. In addition, an optimal environment for the patient is to be created through nutrition, the organization of everyday life and support of the psyche. A balanced diet rich in vitamins helps the organism to deal with the disease. Sufficient exercise and the promotion of joie de vivre are also important in order to improve the overall situation.

Being overweight should be avoided and the child should not be in areas where smoking or excessive alcohol consumption is taking place. The child’s clothing should be adapted to the needs of the body. Sufficient heat supply is particularly important so that no complications arise or existing symptoms increase. If you have a fever, make sure you drink enough fluids. In these cases, the normal daily requirement must be increased.

Still Syndrome