ICD-11 classes
15 Diseases of the musculoskeletal system or connective...
Arthropathies
Inflammatory arthropathies
FA24 — Juvenile idiopathic arthritis
FA24.4 — Juvenile systemic arthritis

ICD-11 FA24.4 — Juvenile systemic arthritis

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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.