Adult onset Still Disease
| Epidemiology | |||||
|---|---|---|---|---|---|
|
Gender ratio |
1 : 1 |
Average onset age |
16-35 y |
Incidence |
1/105 - 106 |
Disease presentation
The disease is characterized by intense systemic inflammation with heterogeneous flare frequency. It is usually diagnosed after the elimination of all other possible causes (infection, autoimmunity, cancer)
Main clinical symptoms
Repeated hyperthermia for a few hours in the evening
Joint pain/swelling
Skin rash
Biological signs
Increase in white blood cell counts
Increase in ferritin
Decrease in glycosylated ferritin
Therapy
- Corticosteroids
- Non-steroidal anti-inflammatory drugs
- Anti-IL-1 (e.g. Anakinra, Canakinumab)
- Anti-IL-6 (e.g. Tocitizumab)
Juvenile onset idiopathic arthritis
| Epidemiology | |||||
|---|---|---|---|---|---|
|
Gender ratio |
1 : 1 |
Average onset age |
1-5 y |
Incidence |
0.5-0.9/105 |
Disease presentation
The disease is characterized by significant extra-articular symptoms and heterogeneous evolution. Some patients will experience no recurrence while other tend to develop polyarticular chronic disease. The diagnosis is based on Edmonton criteria, revised by ILAR.
Main clinical symptoms
Repeated hyperthermia (once or twice daily)
Joint pain/swelling on small or large articulations (maybe delayed and increasing with time)
Skin rash (pink macules)
Biological signs
Increase in ferritin
Decrease in glycosylated ferritin
Therapy
- Corticosteroids
- Non-steroidal anti-inflammatory drugs
- Anti-IL-1 (e.g. Anakinra, Canakinumab)
- Anti-IL-6 (e.g. Tocilizumab)
