In general, it appears between 3 and 5 years. The child declares a fever spike, unexplained by a virus or an apparent event. He gets over 39 degrees for about 24 hours and has a fleeting rash (a rash), pain in some (or all) joints. These symptoms are those of Still’s disease, which in children is calledjuvenile idiopathic arthritis systemic.
What are the clinical manifestations of the disease?
Whether in adults or children, we find these feverish episodes, with peaks especially in the evening, which last a long time (between 1 and 4 weeks, or more), joint pain (arthralgia), an erythematous rash – maculo-papular which does not scratch, salmon-pink in color. There may also be in adults pharyngeal pain, liver damage, increased volume of lymph nodes (adenopathy).
Where does this disease come from? Who does she touch?
Systemic juvenile arthritis is an autoimmune disease, of the inflammatory type, rare. Innate immunity is disrupted, the body produces white blood cells (neutrophils and monocytes) in large quantities, it also faces a surge of cytokines, as if responding to an attack from its immune system. It also does not produce autoantibodies. The causes of Still’s disease are still very unclear: we have not yet isolated genetic responsibility.
For the form that affects minors, it affects both girls and boys and is part of a group of 5 diseases, called “juvenile idiopathic arthritis” (JIA) which concerns between 1.6 and 23 children out of 100,000. Systemic form manifests before 16 years, and represents 10 to 20% of cases of this group of pathologies (JIA).
How does the disease progress? How do we treat it?
“The evolution of the disease varies according to the individual, ranging from a monocyclic form to a predominantly systemic recurrent form, passing through a progressive polyarthritis that can lead to a serious and degenerative joint disease”, notes Orpha.net. But according to the scientific literature on the subject, it is difficult to predict its evolution precisely.
It is not always easy to diagnose: it is estimated that arthritis must last at least 6 weeks to confirm the diagnosis, but sometimes it is absent at the start, emphasizes the Swiss Medical Review. Of course, we first rule out the many diseases that cause similar symptoms, such as malaria, Lyme, mononucleosis, leukemia, lymphoma etc. “Presumptive diagnostic criteria are being developed by a working group of the Pediatric Rheumatology European Society (PReS) in order to allow the diagnosis to be evoked earlier”, specifies Revmed.
Treatments aim to bring systemic and articular manifestations into remission, explains the Swiss Medical Journal. Against mild forms, non-steroidal anti-inflammatory drugs (NSAIDs) can be offered in the first instance, or high-dose corticosteroids, says Orpha.net. A background treatment based on daily corticosteroids can also be put in place. Cytokine inhibitors are also effective.
Sources: Orpha.net, Swiss Medical Journal
