FA24.4 Juvenile systemic arthritis

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2025-01

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.

postcoordination

synonyms

• Juvenile systemic arthritis
• Still disease
• Juvenile systemic onset arthritis complicated by macrophage activation syndrome
• Juvenile systemic arthritis, multiple sites
• juvenile arthritis with systemic onset, multiple sites
• Juvenile systemic arthritis, shoulder region
• juvenile arthritis with systemic onset, shoulder region
• Juvenile systemic arthritis, acromioclavicular joint
• Juvenile systemic arthritis, glenohumeral joint
• Juvenile systemic arthritis, sternoclavicular joint
• Juvenile systemic arthritis, upper arm
• juvenile arthritis with systemic onset, upper arm
• Juvenile systemic arthritis, elbow joint
• Juvenile systemic arthritis, forearm
• juvenile arthritis with systemic onset, forearm
• Juvenile systemic arthritis, wrist joint
• juvenile arthritis with systemic onset, wrist joint
• Juvenile systemic arthritis, hand
• juvenile arthritis with systemic onset, hand
• Juvenile systemic arthritis, carpus
• Juvenile systemic arthritis, fingers
• Juvenile systemic arthritis, metacarpus
• Juvenile systemic arthritis, pelvic region or thigh
• juvenile arthritis with systemic onset, pelvic region or thigh
• Juvenile systemic arthritis, hip joint
• Juvenile systemic arthritis, lower leg
• juvenile arthritis with systemic onset, lower leg
• Juvenile systemic arthritis, knee joint
• Juvenile systemic arthritis, ankle or foot
• juvenile arthritis with systemic onset, ankle or foot
• Juvenile systemic arthritis, metatarsus
• Juvenile systemic arthritis, tarsus
• Juvenile systemic arthritis, toes
• Juvenile systemic arthritis, ankle joint
• Juvenile systemic arthritis, other joints in foot
• Juvenile systemic arthritis, head
• Juvenile systemic arthritis, neck
• Juvenile systemic arthritis, vertebral column

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