Adult-Onset Still Disease: Symptoms, Causes, Treatment
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Adult-onset Still disease is a rare inflammatory disorder causing recurrent fever, rash and joint symptoms. Diagnosis requires excluding mimicking illnesses; one ferritin result cannot confirm it.
What is Adult-Onset Still Disease?
Dysregulated inflammation may cause episodic attacks or persistent activity. Joint and organ involvement varies between individuals.
Related reading: Lupus as a differential diagnosis.
Symptoms
Repeated high fevers, a transient salmon-colored rash, sore throat and painful or swollen joints may occur. Fatigue and enlarged lymph nodes or liver are possible.
Causes
The precise cause is unknown. Pathways involving interleukin-1 and interleukin-6 contribute; the condition is not spread through contact.
Risk factors
Adults of different ages can be affected. Persistent fever or changing blood counts and liver tests require assessment rather than assuming every change is an ordinary flare.
Diagnosis
Evaluation combines the fever pattern, examination, blood counts, inflammatory markers and organ tests. Ferritin is supportive but nonspecific; infection, malignancy and other inflammatory diseases must be considered.
Treatment
Current recommendations support considering IL-1 or IL-6 inhibitors early after diagnosis. Steroids and other therapies are individualized, with an aim to limit prolonged steroid exposure.
Complications
Joint damage and inflammation around the heart or lungs may develop. Macrophage activation syndrome is a severe complication affecting blood cells, liver and multiple organs.
Prevention
There is no established way to prevent onset. Symptom tracking, blood monitoring and appropriate vaccinations support care; do not independently change immunosuppressive treatment.
When to see a doctor
Persistent fever with rapid deterioration, bleeding, confusion or breathlessness needs emergency care. Serious infection or macrophage activation syndrome must be considered.