Still's disease is also referred to as systemic juvenile idiopathic arthritis . This is a rheumatic disease that affects not only the joints but also the organs. The word part juvenile means that this is a childhood disease, affecting 100, 000 children in Europe less than one a year Still's disease. It is therefore a rare disease.
Other variants of childhood rheumatism are more common and occur in about 10 out of every 100, 000 children. Still's disease - also known as "still syndrome" - also occurs in adults and is referred to as adult Still's disease . This also affects less than one per 100, 000 people per year.
With appropriate therapy, Still's disease has a relatively good prognosis, but there are also severe courses.
In all diseases of the so-called rheumatic type - including the Still's disease - typically an inflammatory reaction of various joints occurs. Particularly affected are the larger joints such as the ankle joint, knee joint, hip joint, elbow joint, shoulder joint or wrist.
There is pain, swelling, redness and overheating in the affected joint (s). In the case of children, for example, this can be shown by refusing to walk. In the case of Still's disease, however, these typical joint complaints often do not occur until months after the onset of the disease. This makes the diagnosis difficult.
The disease usually begins with episodes of high fever around 40 ° C, which occurs especially in the morning and evening. The fever can not be lowered sufficiently by conventional antipyretic drugs. Those affected often feel dull and beaten,
Children can be noticed by reduced food and drink, increased need for sleep and increased screaming.
At the beginning, therefore, Still's disease often presents as an acute infection. Accompanying it comes to a salmon-colored fine-blotched rash in the area of belly, chest and back as well as upper arms and thighs. The rash itches frequently. At the beginning of the disease, there may be pain in the area of the cervical spine, which occur, for example, when turning the head.
In Still's disease, inflammatory organ involvement typically occurs. This can manifest itself very differently depending on which organs are affected. There are symptoms such as pain in breathing through inflammatory involvement of the lung, cardiac pain due to inflammation of the pericardium and abdominal pain due to inflammation of the peritoneum.
In addition, there is typically a swelling of numerous lymph nodes distributed throughout the body (generalized swelling of the lymph nodes) and an enlargement of the spleen and liver (hepatosplenomegaly), which may be noticeable on an ultrasound examination of the abdomen (abdominal ultrasound).
It is characteristic of Still's disease that in addition to joint involvement, internal organs are also affected. Various organs can become infected as part of the disease and thus lead to complaints.
The perineal (peritonitis), the pericardium (pericarditis) and the lung pleura ( pleurisy ) are most commonly affected by the inflammatory reaction. Possible symptoms are accordingly abdominal pain, heart trouble or pain during deep inhalation.
Frequently, enlargement of the spleen and liver also occur. However, this increase is not due to a directly inflammatory involvement of these organs.
Rarely, in adult morbidity, an inflammatory involvement of the meninges ( meningitis ) may occur.
There is no causal therapy that can cure Still's disease. However, there are many medications that can help prevent or at least minimize damage to the joints and organs that would be caused by the constant inflammation.
In particular, in children who are still in the growth process, such an anti-inflammatory therapy is essential, otherwise it may lead to permanent movement restrictions or misalignments. But for adults, too, the anti-inflammatory therapy to maintain quality of life plays an important role.
Drugs used to treat Still's disease include non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen, naproxen or diclofenac, glucocorticoids such as prednisolone or so-called basic disease drugs or DMARDs such as methotrexate, sulfasalazine or azathioprine.
Usually, a treatment attempt with NSAIDs is made over the course of a few weeks at the beginning because these are the drugs with the fewest serious side effects. If this does not provide adequate relief, drugs from one of the other groups will be used. Although glucocorticoids are often highly effective, they are given as short a duration as possible in long-term therapy because of their many undesirable effects.
It is often necessary to start with glucocorticoid therapy until the effect of the basic therapies has begun. These have a delayed onset of a few weeks to 3 months. They often need to be given over several years.
As a relative new therapy option for Still's disease, so-called biologics such as anakinra are increasingly coming to the fore. These are antibodies that bind to certain receptors or mediators of the inflammatory response and thus lead to an inhibition of inflammation.
In addition to drug therapy, regular physiotherapy and occupational therapy play an important role, especially in juvenile Morbus Still.
In order to arrive at the correct diagnosis the exact anamnesis, thus the collection history of illness, is crucial. Important here are the symptoms in particular.
There are also various blood tests. A characteristic of a Still's disease is a marked increase in inflammatory parameters in the blood. These include the values CRP and erythrocyte sedimentation rate ( ESR ) as well as the number of white blood cells ( leukocytosis ). In addition, in most cases there is often an increase in the number of platelets ( thrombocytosis ) and anemia ( anemia ).
Typically, antibodies typical of autoimmune diseases are not detectable in the blood. However, these findings are not specific to the disease. There is no specific test that can detect Still's disease. Rather, collecting and evaluating various examinations ultimately leads to the diagnosis.
In the case of the infantile form of Still's disease, the eyes are also examined in detail for the purpose of further diagnosing the disease since an ocular involvement can occur in various types of rheumatoid arthritis which, if left untreated, can lead to loss of vision. Typically, eye involvement in Still's disease can not be demonstrated.
To confirm the diagnosis, further examinations such as ultrasound of the abdomen or ultrasound / X-ray / MRI images of specific joints are possible.
So far, the cause of Still's disease has not been fully understood. It is a so-called multifactorial disease, ie a disease that arises from an interaction of many factors.
It can be assumed that certain genetic conditions increase the risk of developing Still's disease. If other factors are added, the disease breaks out.
The current state of research suggests that Still's disease is an autoimmune disease. This means that the body's immune system falsely attacks structures in the body and thus leads to repeated strong inflammatory reactions.
Life expectancy is usually not impaired by Still's disease, provided that it is adequately treated. With advances in anti-inflammatory and immunosuppressive therapy, the rate of lethal (fatal) complications has dropped significantly in recent decades.
Only rarely there are highly complicating events with fatal outcome.
The disease begins in most cases with recurring attacks of fever and rash as well as fatigue and fatigue. Joint problems often manifest months after the onset of the first symptoms.
The course of the disease and the prognosis are individually very different. In some cases, the disease is completely reduced in childhood and adolescence, while in others it persists throughout life. A distinction is then made between a course in which the symptoms occur in recurring episodes between which symptom-free weeks, months but also years can be and a chronic course in which the symptoms persist permanently.
In about 20-30% of affected patients there is a permanent suppression of the disease (remission). About 40% of sufferers suffer from only mild chronic joint inflammation, which is well-managed under a drug therapy and supportive physiotherapy.
Symptoms of the organs can occur again and again, but also completely absent for years. In addition, there are also cases in which the disease is hardly controllable by the therapy and which is accompanied by an increasing joint destruction and complications in the area of the organs.
A Still's disease can not be cured. At least not in the traditional sense.
There are patients who can be converted to a stage of remission by therapy. Remission in this case means that there are no more symptoms, so the disease is completely under control. However, as it can flare up again at any time, there can be no talk of a cure in the traditional sense.
Yes. There are complications in the disease process that can lead to a fatal outcome.
These include in particular the so-called macrophage activation syndrome, also referred to as hemophagocytic Lymphohistiocytosis. In the context of Still's disease or as part of a viral infection, the immunosuppressive therapy of Still's disease leads to a massive overshooting inflammatory reaction of the immune system.
It comes to persistent fever, spleen enlargement (splenomegaly) and various blood picture changes. The diagnosis is not always easy, because the clinical symptoms mentioned, ie fever and spleen enlargement, also occur in a non-complicating Morbus Still. Only the blood picture changes then point in the direction of a macrophage activation syndrome. Therapeutically, a high-dose cortisone shock therapy, immunosuppressants and biologics are used. The mortality of macrophage activation syndrome is very high without appropriate therapy. But even under therapy, the lethality is up to 40%.