ICD-11 code : FA24.4;
Preferred Label : Juvenile systemic arthritis;
ICD-11 definition : Systemic-onset juvenile idiopathic arthritis represents 10-11% of cases of juvenile
idiopathic arthritis (JIA) and is marked by the severity of the extra-articular manifestations
(fever, cutaneous eruptions) and by an equal sex ratio. Fever peaks are associated
with transient cutaneous eruptions and diffuse erythematosis or urticarial-like lesions.
The presence of arthritis is essential for diagnosis but may appear later in the disease
course. The number of sites affected is variable (mono-, oligo- or polyarthritis)
affecting both the small and large joints in a nearly symmetrical manner. This characteristic
diagnostic triad may also be associated with an adenopathy and hepatosplenomegaly.
Visceral complications (pericarditis, pleural effusion or serous peritonitis with
abdominal pain) may be present.;
ICD-11 synonym : Still disease;
Origin ID : 504387587;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- ICD-10 Mapping
Systemic-onset juvenile idiopathic arthritis represents 10-11% of cases of juvenile
idiopathic arthritis (JIA) and is marked by the severity of the extra-articular manifestations
(fever, cutaneous eruptions) and by an equal sex ratio. Fever peaks are associated
with transient cutaneous eruptions and diffuse erythematosis or urticarial-like lesions.
The presence of arthritis is essential for diagnosis but may appear later in the disease
course. The number of sites affected is variable (mono-, oligo- or polyarthritis)
affecting both the small and large joints in a nearly symmetrical manner. This characteristic
diagnostic triad may also be associated with an adenopathy and hepatosplenomegaly.
Visceral complications (pericarditis, pleural effusion or serous peritonitis with
abdominal pain) may be present.
