juvenile ra

Still’s Disease

Synonym: Juvenile RA,

Juvenile Chronic Polyarthritis, Juvenile Idiopathic Arthritis

April 11, 2023 Ratan M.P.T., (Ortho & Sports) 2

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.


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.

http://en.wikipedia.org/wiki/Chronic_disease

http://en.wikipedia.org/wiki/Chronic_disease

http://en.wikipedia.org/wiki/Psoriatic_arthritis


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.



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.



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.


JRA

CLASSIFICATION

Oligoarticular JRA

Polyarticular JRA

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.

http://en.wikipedia.org/wiki/Antinuclear_antibody


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 continuous fever)

http://en.wikipedia.org/wiki/Fever

http://en.wikipedia.org/wiki/Rash


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, Tenosynovitis, 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, there is some evidence that the two conditions are closely related.

http://en.wikipedia.org/wiki/Internal_organ

http://en.wikipedia.org/wiki/Macrophage_migration_inhibitory_factor

http://en.wikipedia.org/wiki/Adult-onset_Still's_disease


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 in girls, in conjunction with psoriasis,

although joint problems may precede the skin manifestations by several years.

http://en.wikipedia.org/wiki/Ankylosing_spondylitis

http://en.wikipedia.org/wiki/Ankylosing_spondylitis

http://en.wikipedia.org/wiki/Inflammatory_bowel_disease

http://en.wikipedia.org/wiki/Psoriasis


Summary of Symptoms

Joint swellingPainStiffnessLimpingLimited movementSlow movementFeverSkin 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


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 UrinalysisBone marrow examinationThyroid 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.

http://en.wikipedia.org/wiki/Blood_tests

http://en.wikipedia.org/wiki/X_rays

http://en.wikipedia.org/wiki/Antibodies


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.

http://en.wikipedia.org/wiki/Fracture

http://en.wikipedia.org/wiki/Cancer

http://en.wikipedia.org/wiki/Infection


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 RxPhysiotherapySurgical


Medical Treatment

Goal: – Control symptoms, – Prevent joint damage and maintain

functionMedications:

– 1. Non-steroidal Anti-Inflammatory Drugs (NSAIDS) • Motrin or Advil

– 2. Disease Modifying Drugs (DMARDS)• Hydroxychloroquine: Plaquenil• Sulfasalazine: Azulfidine• Methotrexate: Rheumatrex


Treatment: Physical Measures


AimsTo reduce pain & stiffness To minimize swellingTo maintain or increase ROM in affected

jointsTo maintain or increase muscle

strength in affected groupsTo prevent deformitiesTo 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.• Time-2-3 minutes


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 tissueUltrasonic 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 compressionElevation of the limbActive ROM exs.


Manual therapy

Mobilisation – Should be avoided in pain and

swellingManipulationMyofascial releaseTrigger point therapyAcupuncture 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 loose joints, are recommended.


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.


– 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 in gait training


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.

http://en.wikipedia.org/wiki/Sports


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

http://www.v-aldape.com/niki.htm


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 JRA.

juvenile ra

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