Juvenile Idiopathic Arthritis ( JIA)

Prof Deepthi Samarage

 Juvenile Idiopathic Arthritis ( JIA)

• Arthritis present for at least 6 weeks presenting

before 16 years

• Either insidious or abrupt disease onset

• Morning stiffness and pain

• Gelling + ( stiffness after prolonged rest)

 DD
Other causes polyarthritis

 Rheumatic fever

 Reactive arthritis

 SLE

 acute leukaemia
 Systemic-onset JIA
 Oligoarticular JIA( persistent/extended)
 Polyarticular JIA ( Rh Factor + ve /Rh Factor –ve)
 Psoriatic arthritis
 Enthesitis-related arthritis
 Undifferentiated arthritis
 Oligoarticular JIA ( Persistent)
 Four or less joints affected

 Often, only a single joint is involved at onset.

 Knees and ankles are more affected

 If > 4 joints involved after 6 months-

oligoaricular extended JIA

 ANF + ve ( 20%)

 usually Rh Factor Negative

 Uveitis is common

 Prognosis is good
 Systemic JIA

 May present with continued high grade fever

 Rash transient with fever
 Initially may have arthralgia/myalgia and no arthritis -
later develops - polyarthritis
 generalized lymphadeopathy
 hepatosplenomegaly
 Serositis( pleuresy /pericarditis)
 Anaemia
 Thrombocytosis
 High CRP/ESR
 Polyarticular JIA
Rh Factor – ve
Polyarticular JIA

Rh Factor + ve
( older girls - similar to adult
Rheumatoid arthritis)
 Investigations
• ESR or CRP
• FBC
• LFT
• Serum creatinine levels
• Antinuclear antibody (ANA)
• Rheumatoid Factor
• DS DNA
 Psoriatic Arthritis
• Large and small joints

• Psoriasis

• Nail pitting /dystrophy

• Uveitis
 Enthesitis-related arthritis

 Older boys 6-16 years

 Lower limb large joints

 Involvement of sacroiliac joints later

 Enthesitis- tendon/ligaments insertion site inflammation

 Acute uveitis

 HLA B 27 +
 Principles of Management

 Induce remission as soon as possible - drugs

 Multidiciplinary care – with paediatric

rheumatologist/physiotherapist/orthpaedic
surgeon /eye surgeon/social worker

 Education and support

 Physiotherapy to maintain function

 Medical management
 NSAIDs-
( ibuprofen/indomethacin/diclfenac sodium/naproxen )
 Do not use a monotherapy > 2 months

 Intrartcular steroids – usually in monoarticular

 Systemic steroids in systemic JIA/MAS

 Methotraxate( FBC monitoring)

 Cytokine Modulators( Biologics) ( anti TNF α ) adalimumab,

etanercept, and infliximab/ IL1, IL6
 Acute stage complications

 Macrophage Activation Syndrome (MAS)

(Haemophagocytic lymphohistiocytosis)

 Pancytopaenia

 Treated with Steroids/Cyclosporins

 Prognosis
• Good in oligoarticular/poor in Rh Factor + ve
polyarticular

• Better outcomes with newer approaches

• Long term - deformities/leg length

discrepaencies/micrognathia/visual
impairment /fractures from osteoporosis
 Uveitis

Oligoarticular & a positive ANA test –

every 3 months 4 years
every 6 months until at least 7 years
Thereafter they are screened yearly for life.

Systemic JIA RF-positive polyarticular JIA are at very low risk and
are screened yearly
Joint deformities
swan-neck or boutonniere deformities
 Radiographic changes in JIA

– Soft tissue swelling

– Osteopenia or osteoporosis
– Joint-space narrowing
– Bony erosions
– Intra-articular bony ankylosis
– Periosteitis
– Growth disturbances
– Epiphyseal compression fracture
– Joint subluxation
micrognathia