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Juvenile Rheumatoid

Arthritis
Farry

Definition

A group of disorders characterized by


chronic arthritis of 1 or more joints with a
duration of at least 6 weeks

RF only (+) in 5% of all patients Some


authors dont agree the term rheumatoid
juvenile idiopathic arthritis (JIA)

Diagnosis Criteria

Age at onset < 16 y.o


Arthritis of 1 or more joints
Symptom duration > 6
weeks
Onset type, after 6 months
of observation:
Polyarthritic (5 or more joints)
Oligoarthritic (< 5 joints)
Systemic (arthritis and
characteristic fever)
Exclusion of other forms of
arthritis

American College of
Rheumatology

Age at onset < 16 y.o


Arthritis of 1 or more
joints
Symptom duration > 3
months
Type:
Pauciarticular (< 5 joints)
Polyarticular (> 4 joints)
Systemic (arthritis with
characteristic fever)

European League Against


Rheumatism

Epidemiology
Incidence: 3-13,9 cases per 100.000
children per year
Prevalence: 113 per 100.000 children
The most common onset age: between 1
and 3 y.o second peak at 9 y.o
Girls:boys 2:1
50% of all cases are pauciarticular form

Etiology
Exact etiology is still unknown
Factors thought involved in causing JRA:

Autoimmune
Genetic
Infection (Mycoplasma fermentans, rubella virus)

Pauciarticular JRA
Low grade inflammation of 1
or several joints in an
otherwise well child
50% of all pauciarticular
type patients have
symptoms in only 1 joint
Joints affected:

Knee
Ankle-subtalar
elbow

Pauciarticular JRA
Morning stiffness
Joints swollen, minimally tender
ESR and CRP normal / mildly
elevated
Uveitis may be present in 20%
cases
Relatively benign cases
average resolve time in 33
months
Some cases may develop into
polyarticular form

Polyarticular JRA

Joints involved:
Large joints in lower
extremity
Small joints in hands and
feet
Cervical spine
Temporomandibular

Warm, tender, painful on


motion, swollen
ROM almost always
limited muscle spasm,
destruction of articular
cartilage and fibrosis

Polyarticular JRA

Some systemic manifestation


may be present:
Low grade fever
Hepatosplenomegaly
Lymphadenopathy

RF-positive form similar to


adult RA:
Rheumatoid nodules
Erosion of joint surface
Disease course extends into
adulthood

RF-negative form remission


after 10 years in 15-50% cases

Systemic JRA
20% of all JRA cases
Associated with worst long term prognosis
severely damaged joints
Remission in 10 years 29-50%
Many organs may be involved liver, spleen,
pleura, pericardium, and skin uveitis is rare
Febrile course with 1 or 2 daily spikes from
normal to 39C or 40C usually occur late
in the afternoon and rapidly returns to
baseline

Systemic JRA

Skin rash:
Erythematous maculae 25mm on the trunk face,
palms, soles, and proximal
extremities

Hepatosplenomegaly and
lymphadenopathy often
present
Pericarditis and pleural
effusions may occur in
10% cases

Radiographic Evaluation

Earliest changes:
Periarticular soft tissue
swelling
Osteopenia around the
joint
Widening of joint space

Late changes:
Destruction of articular
cartilage
Narrowing of joint
space
Joint subluxation

Laboratory Evaluation
No single or definitive test for JRA
diagnosis made by clinical findings
Anemia, leukocytosis, thrombocytosis,
elevated ESR and CRP
ANA may be (+) in 20% cases higher risk
for uveitis
Synovial fluid:

Elevated WBC with neutrophil predominance


Decreased glucose level

Medical Treatment

NSAID are the mainstay of treatment for most


patients:
Aspirin
Ibuprofen
Tolmetin sodium

In more severe disease not responsive to NSAID


methotrexate given weekly orally or subcutaneously
Intra-articular steroid:

Triamcinolone hexacetonide long duration of action


Given once yearly
May improve synovitis increase ROM
Reduce pannus formation
Have no deleterious effect on cartilage

Physical And Occupational


Therapy

Goals of physical and occupational therapy:


Relieve pain
Increase ROM
Improve muscular coordination

Splinting on the wrists and hands reduce the


tendency toward joint contracture and
subluxation
Physical therapy:
Acute arthritis isometric exercises
Subacute/chronic phase dynamic exercises
repetitive progressive stress with exercises aimed at
specific muscle groups

Orthopaedic Treatment

Chronic joint inflammation


muscle spasm to protect
painful joint from motion
muscle and joint capsule
contracture, disuse osteopenia

Synovectomy indicated when


a trial of medical management
for 6 months has failed
hasnt been shown to alter
long term outcome of joint
disease after synovectomy
recurrent of disease is
frequently reported

Orthopaedic Treatment

Flexion contracture of the hip and knee may


results in loss of walking efficiency
contracture of the knee >15-20
significant loss of walking ability

Surgical soft tissue releases of the hip and


knee may result in long term improvements
of ROM and function

Orthopaedic Treatment

Release of knee flexion contracture:


Best done with prone position
Hamstring and iliotibial band are usually released
If necessary, tendinous portions of gastrocnemius
and posterior capsule are also released
ACL may be cut to correct posterior tibial
subluxation

Postoperative care:
Night splinting in extension position for up to 6
months

Orthopaedic Treatment

Soft tissue release for flexion contracture of


the hip:
Indicated when a significant contracture that
interferes with ambulation persists for >6 months
even after aggressive medical therapy
Psoas and adductor tenotomy

Orthopaedic Treatment

Chronic arthritis
increased epiphyseal
circulation and
cytokine-mediated
growth plate
stimulation growth
disturbances
valgus deformity
epiphyseal stapling
or percutaneous
partial epiphysiodesis

Thank You

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