General Practice Services Committee

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...... 2

History...... 2

Physical Exam...... 2

Myths and Facts about Juvenile Idiopathic Arthritis...... 2

Classification of Juvenile Idiopathic Arthritis (2011)...... 2

Treatment and Management Options...... 2

Referral...... 2

Monitoring and Follow-up...... 2

Quick Links

Click on the following links to quickly access the following sections:

 Jump to History

 Jump to Physical Exam and Red Flags

 Jump to Myths and Facts about Juvenile Idiopathic Arthritis

 Jump to Classification of Juvenile Idiopathic Arthritis

 Jump to Management Options and Medication Options

 Jump to Referral

 Jump to Monitoring and Follow-up, GP Responsibilities and GP Resources

of 17 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

 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. Page 3 of 17 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.

History

History Details Comments Identification Name, age, sex and ethnicity of child Accompanying person at visit Chief Complaint Joint pain History of “PQRST” “Typical” inflammatory joint pain: Presenting P = Provocation, Palliation, Progression  Worse at the beginning of the day Illness  What causes the pain? and better at the end  Alleviating and exacerbating factors?  Improves with heat, movement, hot  Getting worse? Better? Staying the same? showers, activity and anti- inflammatory medications  Improved with analgesics? What kinds? How much? How often?  Pain in one joint may be referred to another i.e. hip pain may be  Prodromal viral illness? referred to the knee  Trauma does not typically cause Q = Quality and Quantity arthritis though in many cases of  What does it feel like? JIA attention to a swollen joint may  How often does it bother you? Be specific i.e. # of be brought on by minor injury times in a day / week.  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? Pain that wakes a child up from sleep is typically a red flag and must raise  Interference with activities i.e. activities of daily living suspicion of more sinister causes of – eating, playing, sleeping, dressing, schooling joint pain such as malignancy and  Ask child to rate pain if possible using visual analog should be carefully investigated. scales or pain scales from 1-10 A variety of conditions other than JIA T = Timing can cause joint pain. (See the  When and how did it start? Differential Diagnosis section)  Acute or gradual onset?  Is the pain better at the beginning of the day or at the end of the day? Associated Morning stiffness or “gelling”?  Where is the child stiff? (specific Symptoms Limping? joints) (Musculoskeletal)  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? Swelling? Modification of movements to accommodate joint pain?  If regression affects more than motor development should raise Regression in motor development in a previously suspicion of a child with global normally developing child? developmental delay Inflammatory vs. mechanical symptoms Page 5 of 17 MSK Initiative – Proposed Learning Session – Resource Toolkit – JIA

History Details Comments Review of  H&N: Fever (and patterns)? Alopecia? Oral ulcers? Head-to-toe approach Systems Rashes? Sicca symptoms (dry eyes, dry mouth)? All symptoms listed may be seen in Dysphagia? Dysphonia? Photosensitivity? conjunction with joint pain in various  CVS/RESP: Chest pain? Shortness of breath? rheumatic diseases or other systemic  GI: Abdominal pain? Change in bowel habits? Blood diseases with musculoskeletal in stools? manifestations and should be screened for when assessing a patient with joint  GU: Dysuria? Hematuria? Changes in menstrual pain pattern?  MSK: Muscle pain? Weakness? Raynaud’s Fever + joint pain  always exclude phenomenon? infection i.e. septic arthritis,  Constitutional: weight loss, changes in appetite, osteomyelitis sleep patterns, energy Other History 1. Travel history (screen child and caretakers) Sick contacts – screen for TB and viral infections which can cause myositis 2. Sick contacts and arthritis

3. Past medical history including medications, Medications: some drugs and immunization status 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, 5. Social history – HEADSSS history in all adolescents psoriasis, inflammatory bowel disease, (ensure confidentiality), pets, recent stressors etc.

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.

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  Special attention to lymphadenopathy, skin performed rashes, organomegaly. 4. All joints should be screened, even if  The Pediatric GALS (Gait, Arms, Limbs, Spine) complaints are referred to only one 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  A knowledge of developmental milestones of the examination 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) Page 7 of 17 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 No swelling FOLLOWING: 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 May be present Locking Absent

Warmth, swelling, May have swelling or Joint exam tenderness tenderness. ESR/CRP Often elevated Normal

Step 2: General differential diagnosis of arthritis in children: what to think about?

a. Factors to consider:

 Infection  Trauma  Orthopedic / mechanical

 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, Orthopedic conditions osteochondritis dissecans Reactive arthritis Juvenile idiopathic arthritis Will often have associated GI symptoms Inflammatory bowel disease but may develop arthritis as first symptom 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 Acute onset with abdominal pain, purpuric Henoch-Schonlein Purpura rash on lower extremities, and arthritis/arthralgia Trauma Hemarthrosis Juvenile idiopathic arthritis Systemic rheumatic disease Systemic lupus, Henoch-Schonlien purpura Inflammatory bowel disease Leukemia, bone or synovial tumors, Malignancy neuroblastoma, others

of 17 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 Often have antecedent viral illness, normal Transient synovitis of the hip labs Legg-Calve-Perthes Minimal pain, may present with limp Pre-teen/teen age, insidious pain and limp, Slipped Capital Femoral Epiphysis often overweight child Pain and stiffness, may or may not have Juvenile idiopathic arthritis other joints involved Reactive arthritis Acute onset after an infection Pain and limp, limited range of motion, risk Osteonecrosis of the hip 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 Pigmented villonodular synovitis, Benign neoplasm hemangioma, synovial tumor Hemophillic arthropathy Rare

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 The absence of pain in joints does not exclude joints, it cannot be JIA. a potential diagnosis of JIA. Arthritis in children is called rheumatoid The correct name for chronic arthritis in arthritis. children is Juvenile Idiopathic Arthritis. Arthritis in children is often not a serious Early diagnosis and initiation of treatment for condition children with JIA is important in ensuring a good outcome.

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 Children are usually ill at time of Arthritis with fever for > 2 wks, presentation quotidian, accompanied by one Critical to rule out infection, or more of the following: Systemic arthritis malignancy before making the evanescent rash, hepatomegaly diagnosis or splenomegaly, serositis, generalized lymphadenopathy Arthritis may not be present initially Arthritis may or may not be painful Arthritis in less than 5 joints in Oligoarthritis Most common is single knee joint the first 6 mo of disease 50% of children develop asymptomatic uveitis Disease flares and remissions Persistent Never more than 4 joints are common More than 4 joints after the first 6 Extension of arthritis can occur at Extended mo of disease anytime Flares and remissions common Arthritis in 5 or more joints with Polyarthritis RF negative negative RF test Disease flares and remissions are common More severe long-term disease Arthritis in 5 or more joints with Polyarthritis RF positive course positive RF test Increased risk of joint destruction Arthritis and enthesitis; or arthritis OR enthesitis with one of the following: sacroiliac joint Enthesitis commonly occurs tenderness or inflammatory around the hips, knees, ankles lumbosacral spine pain, HLA B27 and feet Enthesitis related arthritis positivity, acute symptomatic Small % of children develop uveitis, first degree relative with juvenile ankylosing spondylitis HLA B27 associated disease, or over time onset of arthritis in a boy over the age of 8 years Arthritis and psoriasis; or arthritis with one of the following: Psoriasis may develop years Psoriatic arthritis dactylitis, nail pitting or after initial arthritis presentation oncholysis, or psoriasis in a first degree relative. Child with arthritis who meets Unclassified criteria for more than one category or for no category

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. Methotrexate b. Leflunomide c. Sulfasalazine d. 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…

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.

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.

of 17 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. Administer injectable medications such as methotrexate. b. Engage in monitoring patient for side effects of medications. c. Assist in arranging required community services. d. Assist parents with school issues if necessary. e. 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/MPAP/default.asp?s=1