Pediatric Rheumatology Clinic, Jaslok Hospital, Mumbai, India. *
"Pain is inevitable. Suffering is optional" Anonymous.
Juvenile Idiopathic Arthritis (JIA), previously known as juvenile rheumatic disorder of childhood is characterized by synovitis and systemic features.

Classification of JIA
Chronic arthritis in children represents a heterogeneous group of diseases with unknown etiology. Previously, there were two classification systems for chronic arthritis in childhood. To classify these patients in well-defined diagnostic categories, a task force of the International League Against Rheumatism (ILAR) proposed a new classification with precise criteria with the aim of achieving as much homogeneity within categories as possible.

ILAR Classification
Polyarthritis (> 4 joints in first six months of disease)
  • RF positive
  • RF Negative


  • Persistent (<5 joints in first six months of disease)
  • Extended oligoarthritis (onset is oligoarticular but > 4 joints affected in first year)

Systemic onset (arthritis with typical rash and fever)

Enthesitis related arthritis (arthritis with enthesitis)

Psoriatic arthritis (arthritis with typical skin lesions, a family history of psoriasis, dactylitis or nail abnormalities)

Others (non of above / overlap)

Clinical and Laboratory Features:

Systemic onset Polyarticular RF+ Polyarticular RF- Oligoarticular Extended Oligoarticular

(%) Age






Days to months

later. Mean 3

months. Polyarticular. Large joints

including hip and wrist. Small joints of upper extremity> lower extremity. Cervical spine and t.m. joint less affected.

10 yrs +

F:M 2:1

Small joints of hands and feet,

wrists and

carpals. Ankles and knees also common> 4 joints. Family history of RA in about 25% (may post-date child's illness).

10 yrs -

F> M

Rapidly progressive involvement of > 4 joints. Cervical spine and t.m. joint often involved.

Peak 1-3 yrs

F:M 4:1

Usually 1-2

joints. Rarely 3-4 Knee,

elbow and

ankle common. Cervical spine and t.m. joint rare.


F> M

Onset oligoarticular. By end of first year follows a polyarticular course.
Extra-articular Features Classic quotidian

fever. Well

between spikes

should be present at least 2 weeks as a diagnostic

criterion. Truncal evanescent rash

often labeled as drug rash.

Hepatosplenomegaly, adenopathy, pleuritis and

pericarditis (often silent), rarely

pulmonary, cardiac and neurological

Tenosynovitis common. Subcutaneous nodules on

forearm in

30% Lassitude, fever and

weight loss

Low grade

fever in a

Almost none Almost none
Significant elevation.
Mild elevation Normal to
mild elevation
Normal to mild elevation Mild elevation
ANA Usually negative May be

Positive in a

Positive in 40-75%. Usually in low

dilutions. May develop over

time. Repeat in a year in

Similar oligoarticular onset.
RA Usually negative Strong positive in weeks to

months from

onset. 3

positive results in a year

eliminate other transient positivity states.
Negative Rare (<5%) Rare
Synovial fluid Examination not usually

Examination not usually

Examination not usually


WBC/cu mm polymorph response. Proteins elevated. Glucose within 10% of blood glucose.
Similar to

oligoarticular onset
Outcome Flares may be precipitated by viral and other illnesses. About half

have a

remission at

one year. The rest may have a polyarticular course with

about half of them developing end stage arthritis.
Chronic arthritis. May

require life-

long therapy.
Usually good. Requires aggressive management. Excellent. Majority have

remission in

one year.
More severe

course then

those who

remain oligoarticular. Requires aggressive management.

A. The Conventional Pyramid Approach:
The inverted pyramid approach is preferred to the conventional pyramid approach as early control of inflammation is achieved with a combination of rapid acting anti-inflammatory medications & slower acting second line drugs.

Key Message:

  • Juvenile Idiopathic Arthritis (JIA) is the present terminology for Juvenile Rheumatoid Arthritis.
  • JIA is the most common chronic rheumatic disorder of childhood.
  • International league against Rheumatism has classified this disease entity based on precise criteria.
  • The inverted Pyramid approach is preferred to the conventional pyramid approach so as to achieve early disease control as well as remarkable long-term benefits.
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