1. Clinical Practice Guidelines
for the Management of
Juvenile Idiopathic Arthritis
Author: Juliann M. Trumpower FNP-S
Key Words: Juvenile Idiopathic Arthritis, management, practice guidelines, chronic pain,
adolescents, functional disability
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BACKGROUND
Juvenile Idiopathic Arthritis (JIA) is the most common form of rheumatic disease in children,
affecting more than 300,000 children in the United States. It is an autoimmune disorder thought
to be brought on by a complex interplay of genetic and environmental factors. The disease
includes a heterogeneous group of arthritic conditions that begin before the age of 16 and persist
for more than 6 weeks (Seid, M., 2012).
Joint and musculoskeletal pain is a common presenting complaint in primary care. Typically, the
cause is benign and self-limiting, however, in other cases, such as with JIA, long term morbidity
and mortality is negatively impacted when early detection and treatment is not initiated. Diagnosis
and management of JIA has improved in recent years as a result of increased understanding of the
subtypes and clinical presentation of the disease, along with newer medications and treatments.
Despite these advancements, the response to treatment is as variable as the many subtypes, with
some children achieving clinical remission, while others suffer profound functional disability,
chronic pain, growth disturbances and deformity. (Cartwright, T., Fraser, E., Edmunds, S.,
Wilkinson, N. & Jacobs, K., 2014).
EPIDEMIOLOGY
The incidence of JIA in the United States is 45-50 children per 100,000 and 95-130 per 100,000
worldwide. JIA includes polyarticular, oligo articular, psoriatic, enthesitis,-related arthritis
(ERA), systemic arthritis and undifferentiated arthritis. It does not spare any ethnic background
but afflicts girls three times more than boys (Seid, M., 2012).
RISK FACTORS
First degree relative with JIA- monozygotic twins and siblings of children with JIA have
a 15-30 fold increase risk for developing JIA than the general population
Female (75% of children with JIA)
Maternal smoking
2. Early life infection
Recent infection (thought to initiate or augment JIA, much like with Type 1 diabetes)
Influenza, Rubella and Parovirus B19 have been associated with onset/exacerbation of
JIA
Recent vaccination
Stress (Ellis, J., Munro, J., & Ponsonby, A., 2009).
Medical History
Obtain a comprehensive medical history, including growth and development.
Obtain a thorough family medical history as JIA has been shown to have a
genetic/familial basis
Previous and current medication
Prior hospitalizations or surgeries? If so what and when?
Any drug or environmental allergies?
Any recent infections, trauma or stress?
Any illicit drug, alcohol or tobacco use? What has been used to treat symptoms including
OTC drugs and creams? Any use of Complimentary Alternative Medications (CAM)
including herbal supplements, yoga, acupuncture and hypnosis.
History of Chief Complaint
Age of child?
What are symptoms, when did they start and how long due symptoms last?
Morning stiffness? If yes, how long does it last?
What aggravates/alleviates symptoms?
Any recent illness? Fever? If yes, is fever daily and at what time of day?
Any joint pain and or swelling. Number of joints affected. Heat to joints or erythema
(arthritis rarely causes erythema and if present infectious source should be considered)
Unusual fatigue
Weight loss
Growth disturbances in affected limb
Limp present? In toddler, not pulling to stand on awakening from nap?
School attendance? (Sen, E., Clarke, S., & Ramanan, A., 2014)
Oligo arthritis - Arthritis affecting 1-4 joints in the first 6 months of disease
-Often only one joint involved
-Usually large weight bearing joints (knee/ankle)
-Appear well
-May have limp, limb length discrepancy, and/or muscle atrophy
-Most common form of JIA accounting for up to 60%
-Uveitis not uncommon especially if ANA positive
Polyarthritis -Arthritis in 5 or more joints in the first 6 months of disease
-Accounts for 25-40% of JIA
SUBTYPES OF JUVENILE IDIOPATHIC ARTHRITIS AND CLINICAL PRESENTATIONTable
1
3. -May be Rheumatoid factor positive or negative
-Uveitis uncommon
-Joint contractures of proximal and distal interphalangeal joints
-Deformities (Swan’s neck and Boutonniere deformities)
Systemic Onset -Arthritis with or proceeded by a fever
-Fever may occur 1-2x a day at about the same time (predictable)
-Accounts for 10% of JIA
-Child appears ill
-Generalized myalgia and muscles tender to palpation
-Salmon pink, macular rash often occurs with fever and non-pruritic
-May develop serositis, pleural and/or pericardial effusions
-May complain of SOB or chest pain
-High rate of morbidity and mortality
Psoriatic arthritis -Usually mild in children
-Arthritis precedes psoriasis in 50% of children
-Inflammation and swelling of an entire finger or toe
-Nail pitting or splitting characteristic (70%)
-Mono-articular in 50%, DIP joint involvement 50%
-Tenosynovitis in 30%
-May have disordered bone growth (Beukelman, T., et al., 2011)
Enthesitis-related Arthritis (ERA)
-Arthritis and inflammation of an enthesitis site (the point at which a
ligament, tendon, or joint capsule attaches to bone
-Arthritis or enthesitis with at least 2 of the following: 1) inflammation of
the sacroiliac joints or pain and stiffness in the lumbosacral area; 2) positive blood test for HLA
B27 gene; 3) onset of arthritis after age 6 in males; 4) first degree relative with Ankylosing
spondylitis, ERA, IBD or uveitis
-May develop iritis with acute photophobia and conjunctivitis
-High rate of pain and functional disability
-Symptoms worsen in evening and post-exercise
-Complications include restrictive lung disease aortic insufficiency
(Wwiss, P., Beukelmanm, T., Schanberg, L., Kimura, Y., & Colbert, R. 2012)
Undifferentiated arthritis
-Arthritis manifestations that do not fulfill any 1 category
-Arthritis manifestations that fulfill more than 1 category
Diagnostic Studies
JIA is a clinical diagnosis with complete physical exam critical
Rheumatoid Factor, ANA, HLA- B27 assay help define subtype but are not
diagnostic. Many patients will be negative for these and inflammatory markers
including Sedimentation rate and C-reactive protein.
Radiologic studies including X-rays, MRI and Ultrasound may be useful in
assessing and monitoring joint damage and effusions but are not diagnostic in
early disease
CBC with differential (rule out infection or malignancy)
Liver and kidney function should be checked prior to initiation of treatment
4. Tuberculin skin test prior to immunosuppressive therapies
Serial eye exams are needed to prevent vision loss and assess for uveitis and other
inflammatory eye conditions (Sen, E., Clarke, S., & Ramanan, A., 2014).
Differential Diagnosis
Traumatic- fractures, soft tissue injury, non-accidental injury (child abuse) overuse
injury
Orthopedic-hypermobility-associated, slipped upper femoral epiphysis (SUFE), Perthes’
disease, hip dysplasia, osteochondroses, for example, Osgood-Schlatter disease (tibial
tuberosity), Sinding-Larsen syndrome (inferior patella) or Kohler disease (navicular).
Osteochondritis dissecans, Scheuemann’s disease, spondylolisthesis
Infectious- Septic arthritis, osteomyelitis, discitis, soft tissue infections, psoas abscess,
Lyme disease
Inflammatory- Transient synovitis of the hip, JIA, reactive arthritis, acute rheumatic
fever, systemic lupus erythematosus (SLE), juvenile dermatomyositis, vasculitis such as
Henoch-Schonlein purpura (HSP), and Mixed connective tissue disease (MCTD).
Neoplasia (benign)- bone cyst, chondroblastoma, osteoblastoma
Neoplasia (malignant)- leukemia, lymphoma, neuroblastoma, Ewing’s sarcoma,
osteosarcoma, Langerhans cell histiocytosis
Idiopathic pain- Growing pain/benign nocturnal limb pains (BNLP) of childhood,
diffuse idiopathic pain syndrome (juvenile fibromyalgia), Complex regional pain
syndrome (CRPS)
Other- Rickets, sickle cell disease, fabricated or induced illness, celiac disease
Multidisciplinary Management Team
May include some or all of the following:
Primary Care Physician
Pediatric Rheumatologist
Orthopedic Physician
Palliative Care
Physical Therapy
Occupational Therapy
Social Work
Educational Assistance including Individual Education Plan (IEP)
Psychiatry/Psychology
Music Therapist
Child Life specialist
Pastoral Care
Inpatient Rehabilitation for severe pain/ functional deterioration
Goal of Treatment-
-Control inflammation
- prevent morbidity and mortality
-prevent growth disturbances, joint contractures, joint destruction
-prevent functional disability
-control pain
5. -Improve Health-related quality of life (HRQOL)
-assess and treat anxiety/depression related to disease process
-develop adaptive coping mechanisms
-support patient and family
-treat insomnia
-treat/prevent uveitis and other associated eye disorders
-assess and treat medication side effects (i.e. folic acid deficiency from methotrexate) (Suresh,
S., & Shah, R., 2011).
Medications- See Table 2 for commonly prescribed pharmacological agents used to treat JIA
Nonsteroidal anti-inflammatory drugs (NSAIDS)
Oral Corticosteroids
Intra-articular corticosteroids
Conventional Disease Modifying Rheumatic Drugs (DMARDs)
Other DMARDs
Biologics
Pain management- lidocaine patches, opiates, etc.
Drug Dose Frequency Side effects Monitoring
NSAIDS
Naproxen 10mg/kg/dose BID GI bleed, upset
Ibuprofen 5-10mg/kg/dose 3-4x a day aseptic meningitis
Indomethacin 0.5- BID High side effects
1.0mg/kg/dose
DMARDs
Methotrexate 1.0 mg/kg/dose Weekly Gastrointestinal, hepatic Renal Fx
May be given PO/SQ bone marrow suppression, CBC, LFTs
GI and mouth ulcers, alopecia 4-8 weekly
Sulfasalazine 40-60mg/kg/day BID GI, hepatic, rash, allergic CBC, LFT
Reactions, Steven-Johnson’s Q 3 months
Syndrome, assoc. with Reye’s
Syndrome, contraindicated in
G6PD deficiency
Other DMARDs
Corticosteroids lowest dose possible immunosuppression, osteoporosis infection
(0.3-0.5 mg/kg/day) Daily infection, hyperglycemia, weight
May be given intraarticular gain, straie
1mg/kg for large joints
COMMON PHARMACOLOGICAL AGENTS FOR JIA
Table 2
6. Methylprednisone 30mg/kg IV infusion for severe hypertension, arrhythmia heart rate
Disease flare BP
Leflunomide, cyclosporine Used infrequently in JIA
Hydroxychloroquine, azathioprine
Biologics
Tumor necrosis factor Inhibitors
Etanercept (Enbrel) 0.4mg/kg Weekly rash, autoantibodies
SQ Max 50mg increased infection
Infliximab 6mg/kg/IV 0,2 week, then headache, dizziness
(Remicade) Q 4-6 weeks nasal and throat irritation
Adalimumab 24mg/m2 Q 14 days sepsis, opportunistic infection TB test
(Humira) tuberculosis, lupus like reaction
Interleukin-1 Inhibitors (IL-1)
Monoclonal antibodies
Tocilizumab (IL-6) 8mg/kg/IV every 14 days May take 3 months to work
Rituximab 750mg/m2 weekly x 2 infusion reaction, infections
Costimulation blocker
T cell and antigen presenting
Cell path blocking agent
Abatacept 10mg/kg 0,2 week, then Headache, URI, diarrhea,
(Orencia) max 1 g every 4 weeks fever, pneumonia,
Malignancies
(Beukelman, et. al, 2011)
Physical and Occupational Therapy
Prevent contractures
Provide stretching and disease specific exercises
Return functional abilities
Improve strength
Orthotics and assistive technology (Apti, M., et al., 2014)
Psychosocial Interventions
Psychotherapy-Depression, anxiety, and social withdrawal common. Psychological
therapies may include Cognitive behavioral therapy (CBT), Acceptance-based
therapy (ACT), biofeedback, and hypnosis (Tilberg, A., Weiss, K., Harrison, D., &
Bruce, B, 2014).
7. Assessment and treatment of sleep disturbances (Valrie, C., Bromberg, M., Palermo,
T., & Schanberg, L. 2013).
Educational interventions (school absenteeism common)
Financial resources- Families ability to maintain employment may be impacted by
child’s inability to consistently attend school. Medical care and therapies can be
expensive and may not all be covered by insurance.
Complimentary Alternative Medicine (CAM)
Often based on biopsychological theory of disease (aims to treat mind, body and
spirit) and includes such things as yoga, tai chi and hypnosis
Autoimmune disorders tend to flare in response to emotional stressors
Families may not admit to CAM use for fear of judgement
Essential oils, healing touch, herbal /mineral supplements and dietary changes
(Vinson, R., Yeh, G., Davis, R. B., & Logan, D., 2014).
Table 3. Domains of clinical evaluation of Juvenile Idiopathic Arthritis
Biological Psychological Social
Pain characteristics Mood School attendance
Site/sites Anxiety Social functioning (peer/family)
Time features- onset,
frequency, etc.
Depression Parental behaviors (protective
responsiveness, overbearing,
minimization
Descriptors Cognitions (attitudes, beliefs)
pain catastrophizing
Parent characteristics- age,
educational level, economic status
Intensity (0-10) NRS Sleep Cognitions (catastrophizing,
expectancies
Radiation Behavioral problems Isolation
Precipitating factors Social isolation Inability to participate in sports
and hobbies
Relieving factors loneliness Chronic illness in parent-Parent with
chronic pain
Physical functioning hopelessness Community resources available
Level of disability Resilience factors Ability to participate in activities-
outings, Band, support groups
Implication for Primary Care Providers
Early recognition and treatment of JIA can positively impact the overall morbidity and mortality
associated with this condition. Primary care providers must carefully assess and evaluate
complaints of joint and musculoskeletal pain for rheumatological causes and refer patients for
timely evaluation and treatment. In addition, clinicians with patients with known JIA disease
8. must be on the lookout for common complications including infection, effusions, uveitis,
anxiety, depression and insomnia. As with any chronic condition, the provider may be
instrumental in coordinating care that provides for the physical, psychological, social, and
educational needs of the patient.
9. References
Apti, M. D., Kasapcopur, O, Mengi, M., Ozturk, G., & Metin, G. (2014). Regular Aerobic
Training Combined with Range of Motion Exercises in Juvenile Idiopathic Arthritis.
BioMed Research International, 2014, 1-6. doi:10.1155/2014/748972
Beukelman, T., Patkar, N.M., Saag, K.G., Tolleson-Rinehart, S., Cron, R.Q., Dewitt, E.
M…..Ruperto, N. 2011). 2011 American College of Rheumatology recommendations for the
treatment of juvenile idiopathic arthritis: initiation and safety monitoring of therapeutic agents
for the treatment of arthritis and systemic
Cartwright, T., Fraser, E., Edmunds, S., Wilkinson, N., & Jacobs, K. (2014). Journeys of
adjustment: The experiences of adolescents living with juvenile idiopathic arthritis. Child:
Care, Health and Development, 41 (5), 734-743. doi: 10.1111/cch.12206
Colebatch-Bourn, A., Edwards, C., Collado, P., D’agostino, M., Hemke, R., Jousse-Joulin,
S…..Malattia, C. (2015). FR10503 Eular-Pres Points to Consider for the Use of Imaging in the
Diagnosis and Management of Juvenile Idiopathic Arthritis in Clinical Practice. Annals of the
Rheumatic Diseases Ann Rheum Dis, 74 (Suppl 2).
Ellis, J.A., Munro, J.E., & Ponsonby, A. (2009). Possible environmental determinants of
juvenile idiopathic arthritis. Rheumatology, 49 (3), 411-425. doi:10.1093/rheumatology/kep383
Seid, M. (2012). Predictors of health-related quality of life in children and adolescents with
juvenile idiopathic arthritis. Arthritis Care & Research, 64(5), 652-652. doi:10.1002/acr.21604
Sen, E., Clarke, S., & Ramanan, A. (2014). The child with joint pain in primary care. Best
Practice & Research Clinical Rheumatology, 28(6), 888-906. doi:10.1016/j.berh.2015.04.008
Tseng, A. S., Weiss, K., Harrison, T., Hansen, D., & Bruce, B. (2014). Pain Relief as a Primary
Treatment Goal: At What Point does Functioning and Well-Being Become more Important? A
Case Study of an Adolescent with Debilitating Chronic Pain. Pain Research and Management,
19(4), 219-223. doi:10.1155/2014/745458
Weiss, P.F., Beukelman, T., Schanberg, L.E., Kimura, Y., & Colbert, R.A. (2012). Enthesitis-
related Arthritis is Associated with Higher Pain Intensity and Poorer Health Status in
Comparison with Other Categories of Juvenile Idiopathic Arthritis: The Childhood Arthritis and
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Suresh, S., & Shah, R. (2011). Chronic pain management in children and adolescents. Essentials
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Valrie, C. R., Bromberg, M. H., Palermo, T., & Schanberg, L. E. (2013). A Systematic Review of
Sleep in Pediatric Pain Populations. Journal of Developmental & amp; Behavioral
10. Vinson, R., Yeh, G., Davis, R. B., & Logan, D. (2014). Correlates of Complementary and
Alternative Medicine Use in a Pediatric Tertiary Pain Center. Academic Pediatrics, 14(5), 491-
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