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Musculoskeletal Initiative
Resource Toolkit
Juvenile Idiopathic Arthritis
DRAFT – November 4, 2011
Acknowledgements: Pediatric Rheumatology Group, BC Children’s Hospital, Vancouver, B.C.
MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
Table of Contents
Introduction ................................................................................................................................ 3
History ....................................................................................................................................... 5
Physical Exam ........................................................................................................................... 7
Myths and Facts about Juvenile Idiopathic Arthritis ...................................................................11
Classification of Juvenile Idiopathic Arthritis (2011) ...................................................................12
Treatment and Management Options ........................................................................................13
Referral .....................................................................................................................................15
Monitoring and Follow-up ..........................................................................................................16
Quick Links
Click on the following links to quickly access the following sections:

Jump to History
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Jump to Myths and Facts about Juvenile Idiopathic Arthritis
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Jump to Classification of Juvenile Idiopathic Arthritis
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Jump to Management Options and Medication Options
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Jump to Monitoring and Follow-up, GP Responsibilities and GP Resources
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
Introduction
This toolkit of resource material has been compiled from a number of sources including input
from the Pediatric Rheumatology Group, BC Children’s Hospital, Vancouver, B.C
Purpose of Toolkit
This toolkit is part of the General Practice Services Committee (GPSC) Musculoskeletal (MSK)
Initiative covering four specific MSK conditions – osteoarthritis, rheumatoid arthritis, juvenile
idiopathic arthritis (JIA) and low back pain.
The purpose of this toolkit is to:
 Provide health care providers with guidance about early identification and diagnosis of
juvenile idiopathic arthritis (JIA)
 Facilitate appropriate and timely referrals to Specialists
 Support family physicians in referral and treatment decisions
Goals and Desired Outcomes
The goals and desired outcomes of this toolkit are to:
 Identify children and adolescents with JIA as early as possible in the course of disease
to:
o Decrease pain
o Limit or eliminate disability
o Allow early treatment and improved outcomes
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Assist GPs with assessment of children and adolescents with MSK complaints.
Improve referral patterns for children and adolescents with MSK complaints to:
o Avoid multiple referrals and physician visits without diagnosis
o Support relationship of GPs and pediatricians in referral and shared care
networks
Target Population
The target patient population is children and adolescents who are 16 years of age and younger.
Intended Users of the Toolkit Materials
The intended users of this module are family physicians; however, this module may also be
beneficial to other health care professionals including physical therapists, occupational
therapists, nurses, pharmacists, and child psychologists.
Context and Definitions
Juvenile idiopathic arthritis (JIA) is defined by the International League of Associations for
Rheumatology (ILAR) as arthritis of unknown etiology that begins before the sixteenth birthday
and persists for at least 6 weeks with other known conditions excluded.
Most Canadians do not know that children can get arthritis, yet juvenile idiopathic arthritis is one
of the most common disabling conditions of childhood and can affect children as young as one
year old. It is estimated that approximately 1 out of 500 children have JIA.
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
JIA causes daily pain and difficulty with normal children’s activities such as school attendance,
play and sport. Arthritis in childhood frequently continues into adulthood, with about 70% of
patients continuing to have active disease requiring medication. With delayed treatment, there
is a risk of joint damage which will affect a child for their entire life.
Children with JIA in British Columbia have a significant delay in obtaining a diagnosis, being
referred to a pediatric rheumatologist and beginning appropriate treatment. They are
sometimes not referred by a primary care provider for several months up to a year or more after
the onset of symptoms. As children do not make medical appointments for themselves;
therefore, education for caregivers is essential.
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
History
History
Details
Comments
Identification
Name, age, sex and ethnicity of child
Accompanying person at visit
Chief Complaint
Joint pain
History of
Presenting
Illness
“PQRST”
P = Provocation, Palliation, Progression
 What causes the pain?
 Alleviating and exacerbating factors?
 Getting worse? Better? Staying the same?
 Improved with analgesics? What kinds? How much?
How often?
 Prodromal viral illness?
Q = Quality and Quantity
 What does it feel like?
 How often does it bother you? Be specific i.e. # of
times in a day / week.
“Typical” inflammatory joint pain:
 Worse at the beginning of the day
and better at the end
 Improves with heat, movement, hot
showers, activity and antiinflammatory medications
 Pain in one joint may be referred to
another i.e. hip pain may be
referred to the knee
 Trauma does not typically cause
arthritis though in many cases of
JIA attention to a swollen joint may
be brought on by minor injury
 May result in loss of movement but
no instability or locking
R = Region and radiation
Hip pain may be referred to the knee.
S = Severity
 How does it affect the child’s quality of life?
 Interference with activities i.e. activities of daily living
– eating, playing, sleeping, dressing, schooling
 Ask child to rate pain if possible using visual analog
scales or pain scales from 1-10
T = Timing
 When and how did it start?
 Acute or gradual onset?
 Is the pain better at the beginning of the day or at
the end of the day?
Associated
Symptoms
(Musculoskeletal)
Pain that wakes a child up from sleep
is typically a red flag and must raise
suspicion of more sinister causes of
joint pain such as malignancy and
should be carefully investigated.
A variety of conditions other than JIA
can cause joint pain. (See the
Differential Diagnosis section)

Morning stiffness or “gelling”?
Limping?


Swelling?
Modification of movements to accommodate joint pain?
Regression in motor development in a previously
normally developing child?
Inflammatory vs. mechanical symptoms
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

Where is the child stiff? (specific
joints)
For how long? Morning stiffness
due to inflammation typically lasts
at least 30 mins.
Gelling describes the same
sensation as stiffness after a
period of immobility i.e. watching a
movie
Which joints are swollen?
If regression affects more than
motor development should raise
suspicion of a child with global
developmental delay
MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
History
Details
Comments
Review of
Systems

Head-to-toe approach
All symptoms listed may be seen in
conjunction with joint pain in various
rheumatic diseases or other systemic
diseases with musculoskeletal
manifestations and should be screened
for when assessing a patient with joint
pain

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Other History
H&N: Fever (and patterns)? Alopecia? Oral ulcers?
Rashes? Sicca symptoms (dry eyes, dry mouth)?
Dysphagia? Dysphonia? Photosensitivity?
CVS/RESP: Chest pain? Shortness of breath?
GI: Abdominal pain? Change in bowel habits? Blood
in stools?
GU: Dysuria? Hematuria? Changes in menstrual
pattern?
MSK: Muscle pain? Weakness? Raynaud’s
phenomenon?
Constitutional: weight loss, changes in appetite,
sleep patterns, energy
1. Travel history (screen child and caretakers)
2. Sick contacts
Fever + joint pain  always exclude
infection i.e. septic arthritis,
osteomyelitis
Sick contacts – screen for TB and viral
infections which can cause myositis
and arthritis
3. Past medical history including medications,
immunization status
Medications: some drugs and
immunizations are known to trigger
arthralgias, arthritis and syndromes
suggestive of connective tissue
disease; include complementary and
alternative medications
4. Family history
Family history: emphasis on rheumatic
or musculoskeletal conditions and
autoimmunity i.e. RA, SLE, thyroid,
psoriasis, inflammatory bowel disease,
etc.
5. Social history – HEADSSS history in all adolescents
(ensure confidentiality), pets, recent stressors
HEADSSS:
Home/Education and
Employment/Activities/Drugs/
Sex/Suicide/Safety
o If appropriate, screen for STIs
Draft: April 27, 2011 © Dr. Mercedes Chan
References:
1. Foster, H. and Cabral, D. “Is musculoskeletal history and examination so different in
paediatrics?” Best Practice & Research in Clinical Rheumatology. Vol. 20, No. 2, pp.
241-262, 2006.
2. Goldmuntz, E. and White, P. “Juvenile Idiopathic Arthritis: A Review for the Pediatrician.”
Pediatrics in Review. Vol. 27, No. 4, April 2006.
3. Tse, S. and Laxer, R. “Approach to Acute Limb Pain in Childhood”. Pediatrics in Review,
Vol. 27, No. 5, May 2006.
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
Physical Exam
The following are key points to consider during a musculoskeletal examination in children.
Key Points
Comments
1. Assess general health status: ill or well?


An assessment of general health status is
important: Is the child ill appearing or generally
well-appearing?
If the child appears ill, suspicion must be raised
for conditions which require immediate
assessment and more urgent treatment, such
as infection, malignancy, systemic rheumatic
conditions.
2. Have a child friendly approach

Have a child friendly approach to the
examination. Take some time, watch the child
walk around the room, allow them to sit on the
parent’s lap if necessary.
3. A complete physical examination must be
performed

Special attention to lymphadenopathy, skin
rashes, organomegaly.
4. All joints should be screened, even if
complaints are referred to only one

The Pediatric GALS (Gait, Arms, Limbs, Spine)
is an excellent resource for screening children
for musculoskeletal problems.
This is a quick tool, with a few screening
questions and a brief physical examination
manoeuvres, can be performed on school aged
children and older, and has been shown to pick
up important findings.

5. Developmental norms must be kept in mind in
the examination
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A knowledge of developmental milestones of
infants and toddlers is necessary to interpret
complaints of limp or change in gait.
A child who regresses in developmental tasks,
i.e. begins to crawl after walking, requires
urgent assessment by a pediatrician.
Red Flags
The presence of one or more of the following features should alert the physician to proceed with
a further workup without delay, and to consider a differential diagnosis which includes infection
(sepsis, septic arthritis, osteomyelitis), malignancy or other bone tumors, systemic autoimmune
diseases (systemic lupus, mixed connective tissue disease, inflammatory bowel disease,
vasculitis, etc). Early consultation with a pediatrician or pediatric rheumatologist is suggested.

Child who appears constitutionally unwell (i.e., fever, weight loss, weakness)

Bone pain or significant night pain

Regression of motor milestones

Significant functional disability (i.e., child cannot ambulate, child missing large amounts
of school or activities)
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
Differential Diagnosis
The following steps are meant to assist with differential diagnoses of arthritis in children.
Step 1: Is it arthritis?
a. Definition of Arthritis
 Objective signs of arthritis in at least one joint for more than 6 weeks or longer.
 Present in a child younger than age 16 yrs.
 All other causes for arthritis in a child have been ruled out.
The diagnosis of JIA is clinical. Lab tests do not provide the diagnosis; however, lab tests
are helpful in excluding other causes for arthritis.
b. What is arthritis versus arthralgia?
Arthritis
Arthalgia
Swelling of a joint WITH
TWO OF THE
FOLLOWING:
No swelling
Limited range of motion
Usually not present
Tenderness
May be present
Pain with motion
May be present
Joint warmth
Not present
c. Inflammatory versus mechanical joint problem:
Inflammatory
Mechanical
Onset
Insidious
Abrupt
Gelling phenomenon
Present
Absent/mild
Worse with activity
Absent
May be present
Instability
Absent
May be present
Locking
Absent
Joint exam
Warmth, swelling,
tenderness
May have swelling or
tenderness.
ESR/CRP
Often elevated
Normal
May be present
Step 2: General differential diagnosis of arthritis in children: what to think about?
a. Factors to consider:



Infection
Trauma
Orthopedic / mechanical
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA

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Inflammatory
Malignancy
Hematologic
Genetic
b. Is it monoarthritis?
Diagnoses to Consider
Comments
Septic arthritis
Trauma
Toxic synovitis of the hip
Legg-Calve-Perthes, avascular necrosis,
osteochondritis dissecans
Orthopedic conditions
Reactive arthritis
Juvenile idiopathic arthritis
Will often have associated GI symptoms
but may develop arthritis as first symptom
Inflammatory bowel disease
Benign synovial tumors or hemangioma
Hemarthrosis
c. Is it acute arthritis (present for less than 3 months)?
Diagnoses to Consider
Comments
Septic arthritis
Viral-associated arthritis
Reactive arthritis
Post-strep, post GI infection
Acute rheumatic fever
Relatively uncommon
Serum sickness
Henoch-Schonlein Purpura
Acute onset with abdominal pain, purpuric
rash on lower extremities, and
arthritis/arthralgia
Trauma
Hemarthrosis
Juvenile idiopathic arthritis
Systemic rheumatic disease
Systemic lupus, Henoch-Schonlien
purpura
Inflammatory bowel disease
Malignancy
Leukemia, bone or synovial tumors,
neuroblastoma, others
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
d. Is it a child with hip pain?
Diagnoses to Consider
Comments
Congenital hip dysplasia
Usually seen in infancy
Transient synovitis of the hip
Often have antecedent viral illness, normal
labs
Legg-Calve-Perthes
Minimal pain, may present with limp
Slipped Capital Femoral Epiphysis
Pre-teen/teen age, insidious pain and limp,
often overweight child
Juvenile idiopathic arthritis
Pain and stiffness, may or may not have
other joints involved
Reactive arthritis
Acute onset after an infection
Osteonecrosis of the hip
Pain and limp, limited range of motion, risk
factors are steroid use, sickle cell disease,
Cushing syndrome
e. Is it chronic arthritis (>3 months)?
Diagnoses to Consider
Comments
Infection
Tuberculosis, Lyme disease, osteomyelitis
Juvenile idiopathic arthritis
Rheumatic disease
SLE, MCTD,
Malignancy
Benign neoplasm
Pigmented villonodular synovitis,
hemangioma, synovial tumor
Hemophillic arthropathy
Rare
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
Myths and Facts about Juvenile Idiopathic Arthritis
The following table presents some myths and facts about juvenile idiopathic arthritis that are
important for a GP to know.
Myths
Facts
Children do not get arthritis.
Children do get arthritis. JIA can be serious,
associated with significant pain, disability and
long term dysfunction.
Children will grow out of JIA.
Children rarely ‘grow out’ of JIA.
If a child does not complain of pain in their
joints, it cannot be JIA.
The absence of pain in joints does not exclude
a potential diagnosis of JIA.
Arthritis in children is called rheumatoid
arthritis.
The correct name for chronic arthritis in
children is Juvenile Idiopathic Arthritis.
Arthritis in children is often not a serious
condition
Early diagnosis and initiation of treatment for
children with JIA is important in ensuring a
good outcome.
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
Classification of Juvenile Idiopathic Arthritis (2011)
Accurate classification of JIA subtype is useful in understanding prognosis, choosing therapy,
and planning follow-up. Accurate classification of JIA is best done by a pediatric
rheumatologist.
Subtype
Definition
Clinical features
Systemic arthritis
Arthritis with fever for > 2 wks,
quotidian, accompanied by one
or more of the following:
evanescent rash, hepatomegaly
or splenomegaly, serositis,
generalized lymphadenopathy
Children are usually ill at time of
presentation
Critical to rule out infection,
malignancy before making the
diagnosis
Arthritis may not be present
initially
Arthritis in less than 5 joints in
the first 6 mo of disease
Arthritis may or may not be
painful
Most common is single knee joint
50% of children develop
asymptomatic uveitis
Persistent
Never more than 4 joints
Disease flares and remissions
are common
Extended
More than 4 joints after the first 6
mo of disease
Extension of arthritis can occur at
anytime
Polyarthritis RF negative
Arthritis in 5 or more joints with
negative RF test
Flares and remissions common
Disease flares and remissions
are common
Polyarthritis RF positive
Arthritis in 5 or more joints with
positive RF test
More severe long-term disease
course
Increased risk of joint destruction
Enthesitis related arthritis
Arthritis and enthesitis; or arthritis
OR enthesitis with one of the
following: sacroiliac joint
tenderness or inflammatory
lumbosacral spine pain, HLA B27
positivity, acute symptomatic
uveitis, first degree relative with
HLA B27 associated disease, or
onset of arthritis in a boy over the
age of 8 years
Enthesitis commonly occurs
around the hips, knees, ankles
and feet
Small % of children develop
juvenile ankylosing spondylitis
over time
Psoriatic arthritis
Arthritis and psoriasis; or arthritis
with one of the following:
dactylitis, nail pitting or
oncholysis, or psoriasis in a first
degree relative.
Unclassified
Child with arthritis who meets
criteria for more than one
category or for no category
Oligoarthritis
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Psoriasis may develop years
after initial arthritis presentation
MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
Treatment and Management Options
Although overall management of JIA for a child patient should be directed by a pediatric
rheumatologist, coordinated care between the specialist and GP will be essential.
With an established diagnosis of JIA, the pediatric rheumatologist will prescribe medications
dependent on disease severity, disease subtype and potential for long-term damage and
disability. Medication paradigms have changed over the past 5 years to include newer biologic
agents when required, and earlier disease modifying agents (DMARDs) to provide complete and
early disease control.
A complete listing of all medications used in the treatment of JIA, and treatment paradigms is
out of the scope of this document. Children with JIA who require DMARD, corticosteroid, or
biologic therapy should be under the care of a pediatric rheumatologist and these medications
should be prescribed by them.
Medication Options
1. Nonsteroidal anti-inflammatory drugs (NSAIDs): Most children with JIA will take NSAIDs for
control of inflammation and pain. The most common NSAID used in children with JIA is
naproxen, and it is a safe and effective drug, able to be used over long periods of time.
Although there is a risk for gastrointestinal toxicity (gastritis, ulcer, GI bleed), this risk is far
less than seen in adults taking NSAIDs. Renal toxicity is also less common in children than
in adults.
2. DMARDs:
a.
b.
c.
d.
Methotrexate
Leflunomide
Sulfasalazine
Hydroxychloroquine
Gold is rarely used in children.
3. Corticosteroids: Corticosteroids should be used judiciously in children with JIA, due to the
potential for complications. In general, children with systemic JIA may require oral
corticosteroid therapy to control systemic inflammation, and may require this medication
over long periods of time. Children with other subtypes of arthritis may require short-term
corticosteroid treatment during disease flares.
Intra-articular injections with corticosteroid are a common treatment used for children with
arthritis. Joint injections should be performed by an individual comfortable with injecting the
joints of children, and with conscious sedation and appropriate pain control.
4. Biologic agents: Biologic agents may be an appropriate choice of treatment for children with
arthritis who do not respond to NSAID and DMARD therapy, or who are unable to tolerate
standard DMARD therapy. The choice of biologic agents may depend on JIA subtype and
other patient factors including availability of insurance coverage.
Continued on the next page…
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
Many children have excellent responses to a biologic medication; however, there are
significant potential side effects, including immunosuppression. Biologic agents should be
prescribed by a pediatric rheumatologist, in consultation with parents and the child.
Coordinated care between the pediatric rheumatology team and the GP is necessary for
children taking biologic agents, to ensure careful attention to risks of serious infection.
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
Referral
A referral to a pediatric rheumatologist is warranted in the following cases:
1. Child or adolescent with joint pain, swelling, stiffness, or dysfunction which has lasted
longer than 2 weeks, and is unrelated to trauma.
2. Child with signs and symptoms suggestive of a generalized connective tissue or
autoimmune condition.
Please note:
A child or adolescent who is acutely ill can be seen urgently by phoning the pediatric
rheumatology service directly.
Referrals to the Pediatric Rheumatology Service at BC Children’s Hospital are triaged based on
the referral information. If you feel your patient requires an urgent consultation, you may phone
BCCH and ask to speak to the rheumatologist on call.



The direct line to the BCCH Rheumatology Clinic is 604-875-2437.
The BCCH Rheumatology Clinic Booking Clerk can be reached at 604-875-3678.
The general toll-free line for BCCH is 1-888-300-3088.
There is also a pediatric rheumatologist in Victoria, and one in Penticton as well.
In some cases, a referral to a local pediatrician is a helpful interim step, to ascertain whether the
child requires a pediatric rheumatology referral. The pediatrician may be very helpful in working
through the differential diagnosis of joint pain in children, and is an expert in childhood
development.
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MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA
Monitoring and Follow-up
Children with juvenile idiopathic arthritis will need to have regular follow-up with the Pediatric
Rheumatology Team. These follow-up visits will include assessments by the physician to
monitor response to medications, and by the physiotherapist and occupational therapist to
assess functional status, to prescribe specific exercises if warranted, and to evaluate need for
orthotics. A comprehensive treatment plan comes from each patient assessment.
General Practitioner Responsibilities
The GP will receive a letter from each Pediatric Rheumatology visit, which outlines patient
status and any changes in treatment program. Depending on the specific situation, the
Pediatric Rheumatology team may request the GP to do specific follow-up in between visits
which may include:
a.
b.
c.
d.
e.
Administer injectable medications such as methotrexate.
Engage in monitoring patient for side effects of medications.
Assist in arranging required community services.
Assist parents with school issues if necessary.
Provide appropriate immunizations, or modify standard childhood immunization schedule
as outlined by Pediatric Rheumatology Team.
Additional assistance may be essential for children who live at significant distance from the
Pediatric Rheumatology Team. In those instances, the GP may be asked to have regular
interim follow-up visits with the child and family to include specific musculoskeletal assessment
and review of medical program. Communication between the GP and the pediatric
rheumatology team is essential in these cases.
General Practitioner Resources
More information for GPs on JIA can be accessed through the following organizations:

The Arthritis Society – www.arthritis.ca
o

Need Help Managing Juvenile Arthritis?
http://arthritis.akaraisin.com/Common/Event/Home.aspx?seid=3750&mid=8
Mary Pack Arthritis Program
http://www.arthritis.ca/local%20programs/bcyukon/publications%20and%20resources/M
PAP/default.asp?s=1
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