FA24.4

ICD-11 MMS 2026-01

Juvenile systemic arthritis

Description

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.

Coding note from above levels

Arthritis in children, with onset before 16th birthday and lasting longer than 6 weeks

Index terms

  • Juvenile systemic arthritis
  • Still disease
  • Juvenile systemic onset arthritis complicated by macrophage activation syndrome
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  • 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

Exclusions from above levels

  • 4A41.01 – Juvenile dermatomyositis
  • FA20.0 – Felty syndrome
  • 01 – Certain infectious or parasitic diseases
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  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes
  • DA0E.8 – Temporomandibular joint disorders

Sibling codes

  • FA24.0 – Juvenile idiopathic oligoarthritis
  • FA24.1 – Juvenile idiopathic polyarthritis
  • FA24.2 – Juvenile psoriatic arthritis
  • FA24.3 – Juvenile enthesitis related arthritis
  • FA24.Y – Other specified juvenile idiopathic arthritis
  • FA24.Z – Juvenile idiopathic arthritis, unspecified

Foundation entity

Juvenile systemic arthritis – http://id.who.int/icd/entity/504387587

Allowed postcoordination axes

Part of: FA24: Juvenile idiopathic arthritis › 15: Diseases of the musculoskeletal system or connective tissue