Rare illness could be behind repeated fever without a clear cause
Anyone who has a fever from time to time without finding a cause could possibly suffer from Still's disease. This rare condition is also associated with symptoms such as joint pain and rash. If the Still syndrome is recognized in time, it can be treated well.
First of all, it is only evening fever and a sore throat that makes a Still syndrome noticeable. A volatile, salmon-colored rash is also typical of the rare inflammatory rheumatic disease that affects less than one in 100,000 people. Since the initial symptoms are non-specific and the disease is very rare, it often takes a long time to detect Still syndrome.
Avoid consequential damage
The German Society for Rheumatology (DGRh) explains in a recent announcement that there is no reason to suspect a rheumatic disease until the hand and elbow, ankle and knee become infected.
According to the experts, an early diagnosis is important to avoid consequential damage. If diagnosed in good time, a Still syndrome can be treated with medication today.
"As an inflammatory rheumatic disease, Still's disease can initially be treated with non-steroidal anti-inflammatory drugs and cortisone," explains Prof. Dr. med. Hendrik Schulze-Koops, President of the DGRh and Head of the Rheumatism Unit at the Clinic of the Ludwig Maximilian University in Munich.
Avoid life-threatening complications
But this is not always enough. "However, in one to two thirds of Still's syndrome patients, the disease is more aggressive and progresses chronically," says Schulze-Koops. In these cases, modern biologics are often necessary to avoid permanent damage to the joints or life-threatening complications such as multi-organ failure or a coagulation disorder.
As with all inflammatory rheumatic diseases - even with the much more common and well-known joint rheumatism - the inflammation in Still's disease is not limited to an organ or an organ system, but, according to the experts, can spread to very different areas of the body.
In the course of the disease, the heart, lungs or eyes can also become the target of inflammatory attacks.
Complete freedom from symptoms achieved
According to the DGRh, active ingredients from the class of biologicals for the treatment of Still syndrome have been approved for more than ten years. These are antibodies that specifically intervene in the molecular process of the disease - in the case of Still's disease, they curb the excessive activity of the immune messenger substances IL-1 and IL-6, which are produced in a significantly increased amount by those affected.
"The group of these drugs also includes the IL-1 receptor antagonist Anakinra, who has been available for the treatment of Still's disease for two years," explains the expert.
Studies recently summarized in a meta-analysis published in the journal "Rheumatology" showed that anakinra was very effective: 23 to 88 percent of the child and 50 to 100 percent of the adult patients had complete freedom from symptoms achieved, the additional administration of cortisone could have been dispensed with in many cases.
In addition to Anakinra, canakinumab is another substance from the class of biologicals that has also been approved for the treatment of Still's disease and is very effective, Schulze-Koops adds.
Diagnosis needs to be accelerated
As is known today, the excessive immune events in patients with Still's syndrome are mainly due to the cells of the innate immune system; these are extremely active and multiply rapidly.
“In the laboratory, this is noticeable as a high leukocyte value. In addition to this characteristic, the very high value of the protein formed as an expression of inflammation by the liver, ferritin, in the serum of the patient is one of the few diagnostic markers for Still syndrome, ”says Schulze-Koops.
“However, research into the rare disease is far from over. So the causes of Still's disease are still in the dark, and immune activity could also be examined in more detail - after all, every detail that becomes known increases the chance of further targeted biological agents. Antibodies against the messenger IL-18, for example, are also conceivable, which is also increased ”.
Above all, according to the DGRh president, the diagnosis of Still's disease must be accelerated. It still takes a long time before the disease is recognized correctly, the affected person is referred to a rheumatologist and receives effective therapy.
Ideally, in the event of repeated episodes of fever for which there is no obvious cause, every pediatrician and general practitioner should also consider the possibility of Still's disease and refer the patient to a rheumatologist. (ad)
Author and source information
This text corresponds to the specifications of the medical literature, medical guidelines and current studies and has been checked by medical doctors.
- German Society for Rheumatology (DGRh): DGRh: In case of repeated fever without clear causes also think of Still's disease (accessed: March 1st, 2020), German Society for Rheumatology (DGRh)
- Vastert SJ, Jamilloux Y, Quartier P, et al .: Anakinra in children and adults with Still’s disease; in: Rheumatology, (published: 11/26/2019), Rheumatology