Juvenile idiopathic arthritis (JIA), management in children

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Juvenile Idiopathic Arthritis (JIA) is essentially a diagnosis of exclusion and therefore a high index of suspicion for other differential diagnoses is necessary. The chief differentials for a swollen joint will be infection (septic arthritis or osteomyelitis), malignancy (particularly leukaemia or bone tumours) and trauma.

Note: 

  1. More than one swollen joint will always require rheumatology referral
  2. In an otherwise well chid with swollen joint(s) the most likely diagnosis is JIA
  3. Outcome of JIA is better if treated early ∴ have low threshold for referral to Rheumatology
  4. Beware of labelling all ‘limping’ or non-weight bearing as injury related - some falls are ‘red herrings’. Minor falls are common in children and may be co-incidental.
Algorithm for management of child with suspected JIA in ED

Systemic JIA (SJIA)

This is a serious medical condition that can be life-threatening – however it can be difficult to identify early in the course (as arthritis may not be present initially). It may present in many different ways and a high index of suspicion is needed. The disease is present equally in males and females with no preferential age of onset.

SJIA : Diagnosis

Requires presence of arthritis accompanied or preceded by daily-spiking fever plus one or more of the following:

  • Evanescent non-fixed macular rash (pink rash that characteristically coincides with fever peaks)
  • Hepatomegaly or splenomegaly
  • Generalised lymphadenopathy
  • Serositis

The arthritis is often symmetrical and polyarticular and can be absent at onset and develop during disease course.

Laboratory findings

Patients in whom a diagnosis of systemic onset JIA is being considered are at risk of Macrophage Activation Syndrome – Please do ‘MAS Set’ bloods on Trak and ensure Ferritin processed as urgent (will be significantly elevated).  Please contact on-call Rheumatologist urgently.  Lab findings can vary; inflammatory markers often raised, FBC can range from thrombocytosis and leucocytosis to pancytopenias.  Please see separate MAS guideline for further information.  Please note, normal/low ESR and normal/falling platelets are worrying signs.

Management

Discuss with on-call rheumatologist urgently and arrange admission.

Editorial Information

Last reviewed: 01 September 2019

Next review: 30 September 2022

Author(s): Dr Fiona Russell (Consultant in Paediatric Emergency Medicine)

Co-Author(s): Updating authors: Dr Catriona Anderson (GRID trainee in Paediatric Rheumatology) & Dr Neil Martin (Consultant in Paediatric Rheumatology).

Approved By: Clinical Effectiveness

Reviewer Name(s): Paediatric Clinical Effectiveness & Risk Committee