JUVENILE RHEUMATOID ARTHRITIS

DEFINITION
 a kind of rheumatoid arthritis that affects children usually between six ti sixteen years of age
 also known as juvenile idiopathic arthritis because its cause is unknown unlike adult rheumatoid
arthritis

Predisposing Factors
• An immune system that is too active and attacks joint tissues.
• Viruses or other infections that cause the immune system to attack joint tissues.
• Having certain genes that make the immune system more likely to attack joint tissues.

Clinical Manifestation
• joint pain
• joint swelling
• joint stiffness
• trouble in sleeping
• problems in walking

CLASSIFICATION
1. Oligoarticular JRA, which affects four or fewer joints. Symptoms include pain, stiffness, or
swelling in the joints. The knee and wrist joints are the most commonly affected. An
inflammation of the iris (the colored area of the eye) may occur with or without active joint
symptoms. This inflammation, called iridocyclitis, iritis, or uveitis, can be detected early by an
ophthalmologist.

2. Polyarticular arthritis, which affects more girls than boys. Symptoms include swelling or pain
in five or more joints. The small joints of the hands are affected as well as the weight-bearing
joints such as the knees, hips, ankles, feet, and neck. In addition, a low-grade fever may appear,
as well as bumps or nodules on the body on areas subjected to pressure from sitting or leaning.

3. Systemic JRA, which affects the whole body. Symptoms include high fevers that often increase
in the evenings and then may suddenly drop to normal. During the onset of fever, the child may
feel very ill, appear pale, or develop a rash. The rash may suddenly disappear and then quickly
appear again. The spleen and lymph nodes may also become enlarged. Eventually many of the
body's joints are affected by swelling, pain, and stiffness.

Diagnostic Procedures

To diagnose JRA, the doctor will take a detailed medical history and conduct a thorough
physical examination. The doctor may order X-rays or blood tests to exclude other conditions that can
produce similar symptoms.

Other tests that may be done include:

JEFFREY A. LUCERO 1
III Bachelor of Science in Nursing
Cavite State University
 JUVENILE RHEUMATOID ARTHRITIS

• CBC (complete blood count), a common blood test used to evaluate all the basic cellular
components of blood, including red blood cells, white blood cells, and platelets. Abnormalities
in the numbers and appearances of these cells can be useful in the diagnosis of many medical
conditions.
 Blood culture, a test to detect bacteria that cause infections in the bloodstream. This may be
done to rule out infections.
 Bone marrow examination, a test that allows doctors to look at blood where it's formed (in the
bone marrow) to rule out conditions such as leukemia.
 Erythrocyte sedimentation rate, which checks how rapidly red blood cells settle to the bottom
of a test tube. This rate often increases in people when inflammation is occurring in the body.
 A test for rheumatoid factor, an antibody produced in the blood of children with some forms
of JRA. But it's much more commonly found in adults with rheumatoid arthritis.
 ANA (antinuclear antibody), a blood test to detect autoimmunity. It's also useful in predicting
which children are likely to have eye disease with JRA.
 A bone scan, to detect changes in bone and joints to evaluate the causes of unexplained bone
and joint pain.

MEDICAL MANAGEMENT

Treatment begins with a multidisciplinary approach. A number of factors must be considered

when one plans treatment. The specific diagnosis must be established first. Thereafter, psychosocial
factors should be assessed, and long-term prognoses should be explained and discussed with the parent.

A comprehensive treatment plan involves a therapist, a clinician, and a surgeon working

together. The physician must keep in mind the adverse effects of therapy while starting and maintaining
medical treatment. Because of the special problems encountered in young and unique patient
populations—notably their different functional and rehabilitation needs—surgical options must be
planned well. Results in pediatric patients may vary from those observed in adults with RA.
Given the principles described above, the management of JRA must focus on the following areas :
• Psychosocial factors - Including counseling for patients and parents
• Medical care - Use of nonsteroidal anti-inflammatory drugs (NSAIDs) and immunosuppressants
• School performance - Academic counseling, school-life adjustments, physical education
adjustments
• Nutrition - Particularly to address anemia and generalized osteoporosis
• Physical therapy - To relieve pain and to address range of motion, muscle strengthening,
activities of daily living, and conditioning exercises
• Occupational therapy - Including joint protection, a program to relieve pain, range of motion,
and attention to activities of daily living
• Surgery

Specific indications for referral to a pediatric rheumatologist

JEFFREY A. LUCERO 2
III Bachelor of Science in Nursing
Cavite State University
 JUVENILE RHEUMATOID ARTHRITIS

Referral to a pediatric rheumatologist may be indicated when the following situations arise :
• Unclear diagnosis
• Unexplained fever
• Loss of function
• Regression of physical skills with an inability to attend school
• Normal laboratory findings but clinically significant local signs
• Abnormal laboratory findings but a clinical picture that does not fit that of a
rheumatologic disorder
• Unexplained physical findings of joint involvement, fever, and rash
• Need for diagnostic evaluation and long-term management of childhood rheumatologic and
connective tissue disorders, including JRA and spondyloarthropathies
• Need to confirm the diagnosis listed below and to help formulate treatment:
• Henoch-Schönlein purpura
• Apophysitis
• Reactive (postinfectious) arthritis
• Osteochondroses
• Other diagnoses
• Need to evaluate a diagnostic or treatment plan for an autoimmune disorder associated with
other primary diseases (eg, immunodeficiency, cancer)
• Desire for a second opinion or confirmatory evaluation (eg, when a primary care physician
requests an expert opinion because the family requires information from a subspecialist to cope
with the patient's disease process, to accept the treatment plan, to allay anxiety, and/or to receive
education)

Goals of Medical Therapy

The ultimate goals in managing RA are to prevent or control joint damage, to prevent loss of function,
and to decrease pain. These goals are particularly important in JRA, for which the rate of progression
and the onset of debility can be rapid.
Complete remission is a condition wherein the following criteria are met :
• No inflammatory joint pain
• No morning stiffness
• No fatigue
• No synovitis
• No progression of damage, as determined in sequential radiographic examinations
• No elevation of the ESR and CRP levels

Modes of Medical Therapy

• Rest and splinting: The mainstay of all therapy in the acute phase is rest and splinting to relieve
pain. Splinting may be alternated with traction. These methods not only relieve pain but also
allow for early recovery.

• Range-of-motion exercises and hydrotherapy

 Range of motion should be instituted to maintain nutrition of the cartilage. At the same

JEFFREY A. LUCERO 3
III Bachelor of Science in Nursing
Cavite State University
 JUVENILE RHEUMATOID ARTHRITIS

time, the joint must be splinted in the best possible position to prevent deformities. Any
program must be done within the patient's limits of pain and under the supervision of a
therapist. Exercise preserves joint range of motion and muscular strength, and it protects
joint integrity by providing better shock absorption. Types of exercises that may be
advised include a muscle-strengthening program, range-of-motion activity, stretching of
deformities, and endurance and recreational exercises.
 Nighttime splinting of the hands and feet is often advised.
 Hydrotherapy is a good form of exercise that helps achieve the aforementioned
objectives.

• Pharmacologic management: Patients with JRA have traditionally been treated with a
multitude of drugs ranging from simple NSAIDs to recent immunosuppressants, such as
etanercept. Early intervention is needed to prevent protracted and severe debility in this young
and potentially active age group. Pharmacologic management comprises a combination of
NSAIDs, disease-modifying antirheumatic drugs (DMARDs), and steroids.

• Nonsteroidal anti-inflammatory drugs

 NSAIDs are the mainstays of treatment for JRA. However, their long-term use is
not without adverse effects. In children, NSAIDs are associated with clinically
significant gastric irritation and allergic reactions. These potential effects limit
the use of aspirin as a first-line agent, but it still has its applications. The dosage
of aspirin needed to achieve an anti-inflammatory effect is as high as 80-100
mg/kg/day. The NSAID most commonly and most often given is salicylate. Other
NSAIDs that may be used include naproxen, tolmetin, ibuprofen, and
indomethacin.
 With NSAIDs, the mean time to achieve a response is about 4 weeks, or up to 3
months to achieve a clinically significant improvement in some patients.
Therefore, switching from one NSAID to another without waiting for results to
occur is not a rational approach.
 Foeldvari et al compared celecoxib with naproxen in a multicenter, randomized,
double-blind study of patients with JRA and found that celecoxib dosages of 3
mg/kg bid and 6 mg/kg bid both resulted in better response rates than naproxen
7.5 mg/kg bid. Adverse events were generally the same for all groups, except
gastrointestinal effects were greater with naproxen. The highest response rates
occurred with the higher dose of celecoxib.

• Disease-modifying antirheumatic drugs

 DMARDs, also known as slow-acting antirheumatic drugs, are believed to alter
the course of the disease, as they change its radiologic progression. Double-blind,
randomized, controlled trials have shown 3 DMARDs to be effective in JRA:
methotrexate, sulfasalazine, and etanercept.
 Methotrexate is the most commonly prescribed DMARD. It is more effective in
the oligoarticular variety of JRA than in the systemic variants. Almost 60-80% of
patients taking methotrexate experience some remission.
 Sulfasalazine is effective, but its withdrawal rates are high because of a number
of adverse effects, such as hepatotoxicity and GI irritation.
 Etanercept has been shown to be a potent inhibitor of tumor necrosis factor
JEFFREY A. LUCERO 4
III Bachelor of Science in Nursing
Cavite State University
 JUVENILE RHEUMATOID ARTHRITIS

(TNF), a key proinflammatory cytokine. The use of parenteral etanercept

significantly decreases frequency of disease flares, slowing the radiographic
progression of the disease. It acts for as long as 16 months with sustained
treatment. Infection is a definite risk in the pediatric population. For pediatric
patients, vaccinations should be current before they start etanercept therapy, and
live vaccines should not be given during treatment with etanercept.
 Other DMARDs, such as infliximab and leflunomide, are not well studied, and
additional information is needed.

• Combination chemotherapy: Combination chemotherapy has led to the withdrawal of

drugs after suitable remission is achieved. However, this treatment is not applicable in
JRA, unlike in adult RA, because the prognoses and presentations of patients with JRA
are highly variable.

• Biodrugs
 Some biodrugs, such as anti–TNF, monoclonal antibodies (infliximab,
adalimumab), TNF-soluble receptor constructs (etanercept), and interleukin-1
receptor antagonist (IL-1Ra) have been shown to be effective in RA. Studies
show that for etanercept, the clinical effects are transient; repeated treatments are
needed; and vaccination effects are a risk.
 Lovell et al compared the use of adalimumab with or without methotrexate in
patients with JRA and found that in those patients who did not receive
methotrexate, disease flares occurred in 43% of those who received adalimumab
and in 71% of those who received placebo. In those patients who did receive
methotrexate, disease flares occurred in 37% of patients who received
adalimumab and in 65% of those who received placebo. The response rates were
found to be sustained at 104 weeks following treatment.

Support of patient adherence

• Adherence to therapeutic regimens is a problem in pediatric patients.Adherence to exercise,
medication, and splinting protocols is mandatory for achieving long-term relief from pain and
long-term preservation of joint function.
• To improve patient adherence to treatment protocols, the following measures are recommended:
 Provide education to emphasize the goals of treatment.
 Make treatment regimens as simple as possible.
 Make regimens consistent with the patient's daily routine.
 Offer encouragement to ensure that patients and their caregivers adhere to prescribed
schedules.
 If necessary, enlist the help of a mental health professional if a serious problem is
causing noncompliance.

SURGICAL MANAGEMENT

 Synovectomy
JEFFREY A. LUCERO 5
III Bachelor of Science in Nursing
Cavite State University
 JUVENILE RHEUMATOID ARTHRITIS

The cyclical nature of juvenile rheumatoid arthritis and the possibility of disease flares make the
success of synovectomy difficult to assess. In general, the best results are expected in oligoarticular or
monoarticular disease.
Arthroscopic synovectomy is the method of choice if synovectomy is indicated. It reduces
morbidity and time in bed, and it hastens the patient's rehabilitation and return to normal range of
motion and activity. In addition, arthroscopic synovectomy is associated with less joint stiffness than
open synovectomy.

Ideal indications for synovectomy are the following:

• Involvement of 1 joint or a few joints
• Hyperplastic or wet synovitis
• Failure to respond to nonsurgical treatment
• Minimal or no destruction of the articular surface, with reasonably well preserved joint function

Contraindications to synovectomy are as follows:

• Dry synovitis
• Polyarticular involvement
• Acute phase of the disease
• Systemic disease
• Advanced arthritis

 Osteotomy and Arthrodesis

Osteotomy and arthrodesis are salvage procedures in patients with JRA associated with severe
joint destruction or deformity.
An osteotomy procedure is rarely performed when a deformity affects a joint with fairly well
preserved function and radiologic appearances.
For children with rheumatic disease in the wrist and fingers and in the ankle, arthrodesis is a
good option. This procedure results in a stable and functional joint that can be well loaded and used for
activities of daily living. In this situation, arthrodesis serves a better function than does arthroplasty. In
the hand and wrist, spontaneous fusion tends to occur. Therefore, it is important to ensure adequate
splinting to take advantage of this tendency and, thus, to permit the wrist to fuse in a functional
position.

 Total joint replacement

Total hip and total knee replacements provide excellent relief of pain and restore function in a
functionally disabled child with debilitating disease. However, their role in JRA is fraught with
problems. The patients' young age, short stature, insufficient bone stock, and abnormal bony anatomy
JEFFREY A. LUCERO 6
III Bachelor of Science in Nursing
Cavite State University
 JUVENILE RHEUMATOID ARTHRITIS

all make primary hip arthroplasty challenging. The acetabulum is small and often dysplastic, and the
femur is often narrow and malformed with thin cortices.
Most important among these factors are the recipient's young age, the severe and generalized
osteopenia that is usually present, the patient's poor general condition and compliance, and the small
bones that require small and often custom components. A further complication is the possibility of
reduced survival of the joint replacement because of the patient's age. Still, joint replacement provides
excellent and lasting relief of pain, 2 outcomes that are not achievable with other modalities.

• Total hip arthroplasty

 Williams and McCollough analyzed outcomes after 57 total hip arthroplasties in 34
adolescents (25 female, 9 male; mean age, 16.4 y) with JCA at a mean of 4.7 years.
Most patients received standard Charnley components through a lateral approach; a
few needed custom implants. The rate of revision was high. The rate of radiologic
loosening was 24.6% and rose to 43% in hips followed up for over 5 years.
Symptomatic failure was relatively uncommon, and 2 hips were revised at the end of
5 years. Among 14 loose hips, 2 were symptomatic. The authors thought that the
frequent cortical remodeling they saw indicated the dynamic state of the immature
skeleton, which by itself could cause high rates of loosening.
 Yun et al studied the use of a bipolar prosthesis to treat end-stage juvenile arthritis.
Progressive superior and medial migration occurred despite all augmentation. Thirty-
nine hips were surgically treated in 24 patients, who were followed up for a mean of
12 years. The revision rate was 36%.
 Charnley low-friction hip arthroplasty was studied in a cohort of 186 patients
followed up for a mean of 22 years. Overall survival rates were 91.9% at 10 years
and 83.0% at 15 years. Survival of the femoral component was 95.6% at 10 years
and 91.9% at 15 years, compared with 95.0% and 87.8%, respectively, for the
acetabulum. Only the use of steroids significantly impaired survival.
 Wroblewski et al followed up 85 patients who underwent low-friction hip
arthroplasties and found that 98% were pain free or experienced only occasional
discomfort; 44% had normal or near-normal functioning; and 62% had full range of
movement or close to full range of the prosthetic hip. At 25 years' follow-up,
survival (defined by revision surgery) was 74%.
 Patients younger than 30 years with JRA who underwent hip arthroplasty with
cement were reviewed over 10 years. The 15-year survival rate for their femoral
components was 85%, and that for their acetabular components was 70%, with
revision as the end point. Although the functional results were excellent, survival of
the prosthesis was a cause for concern.
 In patients with JRA, revision total hip arthroplasty is a major undertaking.
Complication rates can be high, even with experienced surgeons. Osteoporosis and
small components are problems in the primary hip. A capacious acetabulum with thin
walls and bone loss, as well as a thin-walled and capacious femur, make revision
difficult and limit the surgeon's ability to achieve secure fixation.
 Cementless hip arthroplasty has superseded cemented hip operations in terms of
long-term viability. Reports about cementless hip arthroplasty in juvenile arthritis
emphasize the need for small components, which limits choices for implants. The
problem of polywear is important because of the small polyethylene liner.
JEFFREY A. LUCERO 7
III Bachelor of Science in Nursing
Cavite State University
 JUVENILE RHEUMATOID ARTHRITIS

 In one series, 34 children with JIA underwent 62 noncemented total hip

arthroplasties. Mean follow-up was 6 years, and the patient's mean age at the time of
index surgery was 18 years. The overall survival rate was 100% for the femoral
component and 90% for the acetabular component. Function at the hip was good, but
this result did not necessarily correlate with an overall functional improvement.
 Uncemented hips are the first choice if the patient's bone stock permits it, and
hydroxyapatite-coated stems have a role in improving biologic fixation. However,
not much information is available about the use of alternative bearings and materials
in this vulnerable patient group.

• Knee replacement
 The knee is the joint most commonly affected in JRA. Knee involvement can
severely restrict activity in adulthood and causes progressive disability.
 In JRA, factors that complicate total knee arthroplasty are the patient's young age,
poor bone stock, and distorted bony anatomy resulting from the effect of the disease
on skeletal growth. Preserving bone stock is a challenge, as is preserving and
recreating a normal bony anatomy. Controversial issues are whether cementation
should be used, whether the posterior cruciate ligament should be retained, and
whether custom or off-the-shelf implants should be used.
 Cemented, off-the-shelf implantation of the total knee was reviewed over 10 years in
12 patients with polyarticular JRA (mean age, 22.4 y). In all patients, off-the-shelf,
cemented posterior-stabilized implants were used. Although the series was short and
only 17 knees were treated, the results were encouraging and comparable to those
achieved with custom implants.
 Tricompartmental knee replacement is a feasible option in knees with clinically
significant disability related to JRA—if the epiphyses are closed and if clinically
significant arthritis is present.
 Routine patellar replacement has been advised in all cases because it provides
excellent relief of pain.
 A 12-year follow-up study of uncemented components showed good results in terms
of pain relief and function. The authors used passive stretch for contractures less
than 10°, custom-made anterior-translation knee braces for contractures between 10°
and 50°, and skeletal traction for contractures greater than 50°.
 Mulhall et al studied 6 patients with JRA who received bilateral combined hip and
knee arthroplasty, 5 of whom were wheelchair bound before surgery (mean age
before surgery, 14 years at hip replacement and 16 years at knee replacement). At
patient follow-up at a mean of 13.8 years for hips and a mean of 17.3 years for
knees, 4 patients were unlimited community ambulators, 1 a limited community
ambulator, and 1 a household ambulator. Revision or loosening occurred in 3 knees
(2 patients) and 5 hips (3 patients).
 Physeal closure, a prerequisite for joint replacement, occurs prematurely in JRA.
Therefore, patients may undergo early knee replacement. Their low levels of
physical activity enhance the longevity of the results.
 Because chronic synovitis occurs, soft tissue releases play an important role. Patients
with severe flexion contractures need relatively extensive resection of the distal
femur, resection of the posterior cruciate ligament, posterior capsular release, and
JEFFREY A. LUCERO 8
III Bachelor of Science in Nursing
Cavite State University
 JUVENILE RHEUMATOID ARTHRITIS

hamstring release during surgery. Valgus deformities need extensive lateral capsular
release and concomitant medial capsular imbrication and advancement. The small
size and dysplastic appearance of the proximal tibia often requires the use of custom
components.

NURSING DIAGNOSIS
1. Pain, acute/chronic
May be related to
Injuring agents: distension of tissues by accumulation of fluid/inflammatory process,
destruction of joint
Possibly evidenced by
Reports of pain/discomfort, fatigue
Self-narrowed focus
Distraction behaviors/autonomic responses
Guarding/protective behavior

2. Mobility, impaired physical/Walking, impaired

May be related to
Skeletal deformity
Pain, discomfort
Intolerance to activity; decreased muscle strength
Possibly evidenced by
Reluctance to attempt movement/inability to purposefully move within the physical
environment
Limited ROM, impaired coordination, decreased muscle strength/control and mass
(late stages)
3. Body Image disturbed/Role Performance, ineffective
May be related to
Changes in ability to perform usual tasks
Increased energy expenditure; impaired mobility
Possibly evidenced by
Change in structure/function of affected parts
Negative self-talk; focus on past strength/function, appearance
Change in lifestyle/physical ability to resume roles, loss of employment, dependence
on SO for assistance
Change in social involvement; sense of isolation
Feelings of helplessness, hopelessness

4. Self-Care deficit (specify)

May be related to
Musculoskeletal impairment; decreased strength/endurance, pain on movement
Depression
Possibly evidenced by
Inability to manage ADLs (feeding, bathing, dressing, and/or toileting)
JEFFREY A. LUCERO 9
III Bachelor of Science in Nursing
Cavite State University
 JUVENILE RHEUMATOID ARTHRITIS

5. Knowledge, deficient [Learning Need] regarding disease, prognosis, treatment, self-care,

and discharge needs

May be related to
Lack of exposure/recall
Information misinterpretation

Possibly evidenced by
Questions/request for information, statement of misconception
Inaccurate follow-through of instructions, development of preventable complications

Nursing Responsibilities
1. Administer analgesics and other medications as prescribed.
2. Teach the client to take medications as ordered and observe for aspirin toxicity and other
adverse effects of the medication.
3. Apply heat and cold compresses, as ordered; heat paraffin from 52 to 54 degrees Centigrade.
4. Promote rest and proper position to ease joint pains.
5. Provide range of motions exercise up to the joint of pain recognizing that some discomfort is
present.
6. Emphasize the need to remain active, but incorporate rest periods to prevent fatigue.
7. Encourage the client to verbalize his/her feelings.
8. Help set realistic goals, focusing on the client's strength.

REFERENCES

ERSTAD, S. 2009. Juvenile rheumatoid arthritis. Retrieved on 30 november 2009 from

http://health.yahoo.com/rheumatoidarthritis-overview/juvenile-rheumatoid-arthritis-topic-
overview/healthwise--hw104393.html

PODUVAL, M. 2009. Treatment for juvenile rheumatoid arthritis. Retrieved on 30 November 2009
from http://emedicine.medscape.com/article/1259999-treatment

JEFFREY A. LUCERO 10
III Bachelor of Science in Nursing
Cavite State University