Definition
-
Rare sickness.
-
Autoinflammatory disease.
-
We speak of early pediatric form if it begins before the age of 16 and of adult form when it begins after 16 years.
-
It can affect several organs.
Epidemiology
-
It is estimated that it affects 1 to 2 people per million French people per year.
-
It affects both men and women equally.
-
It can start at any age.
-
It is estimated that there are between 400 and 700 affected patients in France.
Pathophysiology
-
Not elucidated to date.
-
Is not genetic.
-
Is not of infectious origin and is therefore not contagious.
Clinical
Still's disease is most often manifested by the association of several of the following signs present for 15 to 30 days in the absence of treatment:
-
A high fever, up to 39°-40° often rather at the end of the day.
-
Joint pain or swelling.
-
A rash on the skin often pinkish or salmon pink often during fever peaks.
-
Difficulty/pain when swallowing.
-
Ganglia.
Other rarer manifestations can sometimes be observed:
-
Cardiac involvement: pericarditis (effusion around the heart).
-
Lung involvement: pleural effusion (around the lung).
-
Liver damage: cytolysis, sometimes cholestasis.
-
Rare neurological, ophthalmological or renal damage.
Diagnostic
This is a diagnosis of elimination which requires having previously eliminated an infectious, auto-immune origin and a cancer.
​
It may be useful to use classification criteria such as those of Yamaguchi and those of Fautrel.
Evolution
Perhaps most often as a single push; or otherwise of a chronic evolution with predominance of fever or joint damage, which can lead to the destruction of joints).
Treatment
Several treatments can be proposed. Corticosteroids are most frequently used at the very beginning as well as anti-inflammatories such as aspirin. More recently, first-line anti-Interleukin 1 biotherapies have been proposed, initially favored in children, but their effectiveness is significant with fewer side effects than corticosteroids. In second intention, other biotherapies can be used such as anti-interleukin 6, anti JAK. Methotrexate is regularly given for cortisone sparing purposes.