Juvenile ra

definition

Juvenile Rheumatoid Arthritis (JRA) is
defined as a chronic condition causing
joint
inflammation for at least 6 weeks in a
child
16 years of age or younger.

June 22, 2012 Ratan M.P.T., (Ortho & Sports) 2

introduction

 JRA is a term used to describe a common type of
arthritis in children.
 JIA is a subset of arthritis seen in childhood, which
may be transient and self-limited or chronic.
 It differs significantly from arthritis commonly seen
in adults (OA, RA), and other types of arthritis in
childhood which are chronic
conditions (e.g. psoriatic arthritis and AS).
 It is an autoimmune disorder resulting in joint pain
& swelling.

introduction cont…

 It is an inflammatory condition occurs during
childhood or adolescence & affects one or more
joints, although it can also affect other organ
systems (particularly the eyes).
 It tends to affect major joints rather than smaller
joints of hands & feet as with primary chronic adult
form.
 Atlantoaxial subluxation can be a concomitant
problem.
 The course of the disease is very variable & the
prognosis is good (particularly if only a small
number of joints are involved) in 80% of cases.

Historical background,
occurrence
 The disease was described by G. F. Still in
1897.
 The incidence of JRA is approx. 3–5 new cases
per 100,000 children under 15 years of age.
 JRA usually occurs before age 16 &
symptoms may start as early as 6 months
old.
 Substantial geographical differences exist, with
illness occurring more frequently in northern
countries.
 The male to female ratio is 1:2.5.

Etiology

 It is UNKNOWN but immunological, genetic,
climatic, infectious & psychological factors are
propose etiological factors.
 Immune system:
– Some children with JRA, particularly severe
forms, show anomalies of the immune system
• e.g. antinuclear antibodies or hypogamma-
globulinemia.
– Autoantibodies, abnormal antigen-antibody
complexes & other anomalies detectable in the
lab also occur.

etiology cont…

 Genetic & Climate components:
– There is a North-South differential in the frequency of
the disease which is associated with climate.
– The condition is also widespread in those hot
countries like New Zealand, Australia.
– Common in UK with its damp, cold climate
 Infection:.
– Microorganisms such as Chlamydia
trachomatis, Yersinia enterocolitica and Mycoplasma
fermentans have also been discussed as the possible
cause of JRA.
– A bacteria-specific, synovial cellular immune
response has been observed.

Etiology cont…

 Psychological factors:
– It play a role in the manifestation of disease, as
children have often reported as being in stressful
situation prior to its onset.
– Children with JRA also tend to be rather reserved and
seem to have difficulty in expressing their problems &
conflicts.


Pathology

JRA is generalized disorder of
connective tissue affecting –
– Articular structure &
– Extra articular structures


Articular Changes

Stage I:
– Inflammation of the synovial membrence
spreads to articular cartilage & other soft
tissues.
– Limitation of joint movt with pain & muscle
spasm


Articular changes cont…

Stage II:
– Granulation tissue formation within
synovial membrence & spread to
periarticular tissue.
– Cartilage disintegration & joint filled with
granulation
– Thickening of joint capsule, tendon (with
sheaths) & impaired joint movt
permanently.

Articular Changes cont…

Stage III:
– Granulation tissue converted into fibrous
tissue with adhesion formation between
tendon, joint capsule & articular surface.
– Articular surface cover partly by cartilage
& partly by fibrous tissue.


articular changes cont…

Stage IV:
Permanent joint damage and deformity

Disability


Extra articular changes

Nodule formation:
– In the pressure area & may be
subcutaneous or intracutaneous.
– They may present in organs such as lung
& heart.
Vascular changes:
– It constitute inflammation of all size
arteries.
– The lumen of small vessels can become
obliteration.

Oligoarticular
JRA

JRA
Polyarticular JRA
CLASSIFICATION

Systemic JRA


Oligoarticular (or
pauciarticular) JIA
 Oligoarticular is used with JIA terminology, and
pauciarticular is used with JRA terminology.
 It affects 4 or fewer joints in first 6 months of
illness.
 Oligoarticular JIA –
– Often ANA positive, when compared to other types of
JIA.
– Accounts for about 50% of JIA cases.
– Usually involves the large joints such as the knees,
ankles & elbows but smaller joints (such as the
fingers and toes) may also be affected.

 The hip is not affected unlike polyarticular JIA.
 It is usually not symmetrical
 Length discrepancy & muscles atrophy often
happens which leads to asymmetric growth and
risk of flexion contracture.
 Early childhood onset are at risk for developing
a chronic iridocyclitis or an anterior uveitis
(inflammation of the eye).


 This condition often goes unnoticed; therefore
these children should be closely monitored by
an ophthalmologist.
 If ANA+, patient need routine eye exam every 3
months.
 If ANA- and older than 7 years old, can have
eye exam every 6 months.
 Late childhood onset are at risk for sacrolitis and
spondyloarthropahty.


Polyarticular JRA

 Affecting 5 or more joints in first 6 months of
disease.
 This subtype can include the neck and jaw as
well as the small joints usually affected.
 It is more common in girls than in boys.
 Usually the smaller joints are affected in
polyarticular JIA, such as the fingers and
hands, although weight-bearing joints such as
the knees, hips, and ankles may also be
affected.

 The joints affected are usually symmetrical
 Children with polyarticular JIA are also at risk for
developing chronic iridocyclitis or uveitis and
should also be monitored by an ophthalmologist.
 Rh factor may be positive in polyarticular JIA
and is rarely positive in children with systemic
JIA.


Systemic JRA

 It is also known as "systemic onset JRA”.
 Characterized by arthritis, fever & a salmon
pink rash.
 It affects males and females equally
 It generally involves both large & small joints.
 Systemic JIA can be challenging to diagnose
because the fever and rash come and go.
 Fever –
– Can occur at the same time every day or twice a day
– Often in late afternoon or evening with spontaneous
rapid return to baseline (vs. Septic Arthritis of

 The rash –
– Often occurs with fever.
– It is a discrete, salmon-pink macules of different
sizes.
– It migrates to different location on skin, rarely
persists in one location more than one hour.
– The rash commonly seen on trunk and proximal
extremities or over pressure areas.


 Systemic JIA may have internal
organ involvement:
– Hepatosplenomegaly, Lymphadenopathy, Hepatitis, T
enosynovitis, etc.
 A polymorphism in macrophage migration
inhibitory factor has been associated with this
condition.
 It is sometimes called "adolescent-onset Still's
disease", to distinguish it from adult-onset Still's
disease.
 However, thereRatan some evidence that the two 24
June 22, 2012 is M.P.T., (Ortho & Sports)

Other types OF JRA

 Some doctors include two other, less common
forms:
– Enthesitis-related arthritis & Psoriatic JIA.
 Enthesitis –
– It is an inflammation of the insertion points of the
tendons.
– This form occurs most often in boys older than girls,
characteristically causes back pain, and is linked
to ankylosing spondylitis and inflammatory bowel
disease.
 Psoriatic JIA –
– Often
June 22, 2012 in girls, in conjunction with psoriasis, although
Ratan M.P.T., (Ortho & Sports) 25

Summary of Symptoms

 Joint swelling
 Pain
 Stiffness
 Limping
 Limited movement
 Slow movement
 Fever
 Skin rash

Complications

 Wearing away or destruction of joints
 Slow rate of growth
 Uneven growth of an arm or leg
 Loss of vision or decreased vision from chronic
uveitis (may be severe, even before arthritis is
not very severe)
 Anaemia
 Swelling around the heart (pericarditis)
 Chronic pain
 Poor school attendance & Sports)
June 22, 2012 Ratan M.P.T., (Ortho 27

Investigations

 Acute phase reactants (APRs)
 Erythrocyte sedimentation rate (ESR)
 C-reactive protein (CRP)
 Full blood count (FBC)
 Rheumatoid factor (RF)
 Antinuclear antibody (ANA)
 Urea & electrolytes (U&E)
 Liver function tests (LFT)

investigations cont…

Uric acid/ Synovial fluid analysis
Urinalysis
Bone marrow examination
Thyroid function (TSH, T3,T4)
Hepatic enzymes
(SGOT, SGPT, alkaline phosphatase)
Muscle enzyme (CPK,)

Differential diagnosis

 Joint effusions occur in a range of diseases
– e.g. hemophilia or suppurative arthritis, but also
in other rare conditions such as
enthesopathic arthritis , leukemia , systemic
lupus erythematodes and rheumatic fever .
 A traumatic cause must also be ruled out.
 As tumor-like lesions, pigmented villonodular
synovitis and synovial chondromatosis can also
produce chronic effusions.


Diagnosis

 Diagnosis of JIA is difficult because joint pain in
children can be from many other causes.
 There is no single test that can confirm the
diagnosis and most physicians use a
combination of blood tests, x rays and the
clinical presentation to make an initial diagnosis
of JIA.
 The blood tests measure antibodies & Rh factor.
 Unfortunately, the Rh factor is not present in all
children with JIA.

diagnosis cont…

 X rays are obtained to ensure that the joint pain
is not from a fracture, cancer, infection or a
congenital abnormality.
 In most cases, joint fluid is aspirated &
analysed.
 This test often helps in making a diagnosis of
JIA by ruling out other causes of joint pain.


Diagnostic Criteria of JRA

Onset before age 16 years;
Arthritis involving one or more joints or
presence of at least two of the
following findings:
– Limitation in ROM
– Tenderness or pain with joint movement
– Increased fever
 Disease persisting 6 weeks or longer

Treatment

Medical Rx
Physiotherapy
Surgical


Medical Treatment

 Goal:
– Control symptoms,
– Prevent joint damage and maintain function
 Medications:
– 1. Non-steroidal Anti-Inflammatory Drugs
(NSAIDS)
• Motrin or Advil
– 2. Disease Modifying Drugs (DMARDS)
• Hydroxychloroquine: Plaquenil
• Sulfasalazine: Azulfidine
• Methotrexate: Rheumatrex

Treatment:
Physical Measures


Aims
 To reduce pain & stiffness
 To minimize swelling
 To maintain or increase ROM in affected
joints
 To maintain or increase muscle strength in
affected groups
 To prevent deformities
 To rehab the child to be independent and
educate parents in the management of the
condition

Heat application

Acute conditions –
– Heat application to the inflamed joint is
not recommended.
Chronic conditions –
– Thermotherapy, especially paraffin baths
combined with ex, should included as an
intervention to improve ROM & decrease
pain & stiffness.


Therapeutic ultrasound

 Therapeutic US is effective for reducing joint
tenderness caused by JRA.
 Continuous US is more effective for patients
with chronic JRA.
– Mechanical effect of pulsed & continuous US
increases skin permeability, thus decreasing
inflammatory response, reducing pain &
facilitating soft tissue healing.
– Dosage for acute condition-Initial stage
• 0.25 to 0.5w/cm sq.
•
June 22, 2012 Time-2-3 minutes
Ratan M.P.T., (Ortho & Sports) 39

Therapeutic us cont…

 Failure case
– 0.25 to 0.5w/cm sq.
– Time-4-5 minutes
 Chronic condition
– Maximally up to 2w/cm sq.
– Time-8 minutes
 Ultrasonic 3MHZ-Superficial tissue
 Ultrasonic 1MHZ-Penetrate deeply

Interferential therapy

 It helps in minimizing pain in JRA
 The electrodes needs to place carefully
 Skin care taken in pts with high dose steroid
 Used of such modalities may addicted to the
patient & when experiencing multiple joint pain
it would be impractical.
 Dosage:
– 90 – 100 Hz – reduce nerve accommodation
– 50 – 100 Hz – improve healing, blood supply &
membrane permeability

To Minimize Swelling

Cryotherapy with compression
Elevation of the limb
Active ROM exs.


Manual therapy

Mobilisation
– Should be avoided in pain and swelling
Manipulation
Myofascial release
Trigger point therapy
Acupuncture and
Massage

Exercise

 Target:
– Neck
– Shoulder
– Elbow
– Wrist & hand
– Chest
– Hip
– Knee
– Lower leg

Positioning & Exercise

 Each jt. should moved actively through full
range
 Strengthen the extensor muscels in prone &
supine position


 Shoulder-
– Girdle exercise with breathing exs. will keep the
shoulder & costovertebral jt. mobile
 Elbow-Full flexion of this joint is important for
maintenance of activities of daily living.
– AROM ex are recommended
– Holding arms at full extension (sitting & standing)
– Use of night splints (especially when flexion
contracture begins to develop)
– Extensor muscle strengthening ex.

 Wrist-
– Loss of extension and ulnar deviation at the
radiocarpal joints are often the first limitations
noted.
– A night resting splint is recommended in addition
to the active extension exercises.
 Fingers-
– Terminal flexion and extension are limited
– AROM & PROM exs, preserving muscle power
with squeezing a sponge and not allowing excess
load on the looseM.P.T., (Orthoare recommended.
June 22, 2012 Ratan joints, & Sports) 47

Hip-
– Primarily extension & IR are limited.
– To prevent these pathologies, it is
recommended that patients sleep in the
prone position 2 times per day for 30-min
durations
– Sleep in the prone position at night, and
stretch and strengthen the extensor
muscles.

Knee-
– Extension & flexion limitations are often
observed.
– Night splints should be applied in case
flexion contractures begin to develop.
– Recommended activities include
• Swimming,
• Ascending & descending stairs
• Kicking a ball.

 Ankle-
– In neutral position for heel strike & orderly walking
pattern.
– Wearing appropriate shoes & slightly raising the heels
relieve pain and provide a comfortable walking
environment
 Foot-
– Small, wide feet with high arches, due to premature
closure of tarsal and metatarsal joints.
– This may limit pronation & supination of the mid-foot.
– Plantar fascia can tighten & metatarsal adduction can
be observed. Ratan M.P.T., (Ortho & Sports)
June 22, 2012 50

– To retain flexibility, active & passive ROM
exs
– Picking up marbles from the carpet to
strengthen intrinsic foot muscles
– Using an arch support in the shoes are
recommended.
– Shoes with thick soles and ankle supports
are recommended for these patients.


Hydrotherapy

 All active exs. should be done in full range in
Hydrotherapy pool
 Due to buoyancy providing weight
relief, reeducation of walking can be given
 Passive stretching of tight structures is less
painful in pool.
 Games and activites can encourage children
to move stiffer jts.without their realizing it


Gait Training

 Walking is started in Hydrotherapy pool
where pain relief and increas jt.mobility
allows improvement in gait pattern
 Hip and Knee extension is encouraged
during stance phase together with the push-
off and heel strike at the beginning and end
of the swing phase
 A walking aid may be necessary if child is
limping
 Body weight supported treadmill is helpful in53
June 22, 2012 Ratan M.P.T., (Ortho & Sports)

Surgery

Rarely used in the early course of
disease
Indications:
– Relieve pain
– Release joint contractures
– Replace a damaged joint


Prognosis

 The prognosis depends on prompt recognition &
Rx.
 With proper therapy, some children do improve
with time and lead normal lives.
 However, severe cases which are not treated
promptly can lead to poor growth & worsening of
joint function.
 The greater the number of joints affected, the more
severe the disease and the less likely that the
symptoms will eventually go into total remission.

Prognosis cont…

 Finally, it is important for both the child and
family member to be educated about the
disorder.
 The more educated the person, the better the
care you can receive.
 Chronic JIA is no longer the dreaded disease
where one remains home bound.
 Many children with JIA have gone on to play
professional sports and have a variety of
successful careers.

Frequency

 In the U.S.
– 10-20 cases per 100,000 children
– Pauciarticular and polyarticular disease occur
more frequently in girls
– Both sexes are affected with equal frequency in
systemic-onset disease

 Internationally
– Occurs more frequently in certain populations
(e.g., Native Americans) from areas like British
Columbia and Norway

Mortality

 Less than 1%
 Often associated with
the evolution of
disease to
manifestations of
other rheumatic
diseases


Morbidity

 Morbidity:
– Relates to adverse effects of
medications, particularly NSAIDS
• Abdominal pain due to gastritis or ulcer
disease, hepatotoxicity, renal toxicity

 Psychological Morbidity:
– Situational depression
– Problems functioning in school


Is There a
Bright Side?
 There are
numerous
resources for
parents:
– Websites
– Support systems for
children
– Ongoing research to
improve quality of
life 5-year-old Katie Tortorice leads an
active, healthy life despite having
June 22, 2012 Ratan M.P.T., (Ortho & Sports) JRA. 60

Juvenile ra

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