Department of Paediatrics Clinical Guideline

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1 Department of Paediatrics Clinical Guideline The child and young person with possible arthritis (joint swelling and/or pain, loss of function for >4 weeks) Definition: Juvenile Idiopathic Arthritis (JIA) is defined as arthritis in 1 or more joints for 6 continuous weeks occurring before 16 years of age, after the exclusion of other causes Prevalence: 1:1000 Classification (ILAR 2001): Systemic Onset JIA [SOJIA] (10%) Oligoarticular - 4 joints (60%) o persistent (60%) o extended i.e. > 4 joints involved after 6 months (40%) Polyarticular > 4 joints (20%) o Rf negative (90%) o Rf positive (10%) Enthesitis Related Arthritis (includes Ankylosing Spondylitis) (5%) Psoriatic Arthritis (5%) Unclassified (meets criteria for 2 or more of above) Diagnosis: JIA is a clinical diagnosis based on a full history and examination Differential Diagnosis: JIA is a diagnosis of exclusion and other conditions need to be ruled out such as: Malignancy Acute/chronic bone and joint infections Streptococcal infection Lyme disease Reactive Arthritis Inflammatory Bowel Disease SLE / Juvenile Dermatomyositis / Vasculitis / Sarcoidosis Bleeding Disorders NAI Trauma Hypermobility Growing pains / Biomechanical Pain TB Reactive Arthritis: typically follows a gastroenteritis or urethritis, is more common in males 13 years and over, is self limiting and tends to resolve by 6 weeks Y:\docsdata\paed\Guidelines_Paediatrics\Arthritis Jul 2014.doc Page 1 of 5

2 Presenting Complaint: A patient with JIA more commonly presents with joint swelling and/or loss of function/gait disturbance History of JIA: Joint pain may be absent or mild in proportion to the amount of inflammation and has a high negative predictive value of inflammatory rheumatic diseases in children Isolated musculoskeletal pain in the absence of other signs or symptoms is almost never a presenting complaint of JIA Malignancy should be considered in any patient presenting with musculoskeletal pain out of proportion to clinical findings as pain associated with malignancy tends to precede arthritis by weeks or months. Night pain especially if asymmetrical should also alert to a malignancy. Early morning stiffness / gelling with inactivity Preceding viral illness, sore throat, gastroenteritis, urethritis Exposure to TB /family history of TB Difficulty chewing food Difficulty with fine motor, self help skills Constitutional symptoms fever, weight loss, rash, pallor, mucosal ulcers, fatigue, easy bruising / bleeding Eye symptoms Activity in school and at home Family History of rheumatological diseases Presenting features in the toddler: o Unsettled with early morning nappy change o Refusing to weight bear o Walking with a limp o Delayed motor milestones Examination: Physical findings take precedence over laboratory results A full general and musculoskeletal examination is a must including Skin Hair Eyes Mucous membranes Nails Focal bony tenderness suggests an orthopaedic cause or malignancy Lymphadenopathy, hepatosplenemegaly suggest malignancy, SOJIA, SLE Y:\docsdata\paed\Guidelines_Paediatrics\Arthritis Jul 2014.doc Page 2 of 5

3 Pitfalls in the Diagnosis of JIA: Inadequate history taking Failure to examine the child fully including the entire musculoskeletal system Referred pain e.g. hip to knee, costovertebral to sternum Uveitis with silent arthritis Earache with TMJ joint arthritis Infections Malignancy mimicking JIA Discitis Inflammatory Bowel Disease Investigations: Laboratory investigations are not diagnostic of JIA but are necessary to exclude differentials The following list of investigations is recommended if the diagnosis of JIA is likely but can be tailored depending on history and examination findings: As a minimum all patients should have FBC, film, ESR, CRP Other bloods to consider: U&Es, LFTs, TFTs, ANA, Autoantibody screen, Igs, Coeliac screen, Lyme serology, Mycoplasma serology, Parvovirus serology, CK, LDH, Urate, ASOT, Anti DNAse B, C3, C4, Anti CCP antibodies, HLA B27, VZV antibodies, ACE, urinary HVA and VMA, Quantiferon, Mantoux, ANCA If SOJIA is suspected (fever, rash, lymphadenopathy, organomegaly, weight loss) please include : clotting, fibrinogen, ferritin, lipids, D-dimers Hb : if anaemia suggests SOJIA, malignancy, SLE WBC : if low / norm in child with fever think malignancy, in adolescent think SLE absolute lymphopenia in SLE Plt : Low / norm Plt with ESR / CRP think SLE or malignancy ESR : reflects activity in SLE / JIA but may be normal in JIA The ANA and Rheumatoid Factor tests have a low PPV and are not recommended as routine in the initial investigations of arthritis ANA : up to 30% healthy child are +1:40 and 5% 1:160 Rf is also present in a small percentage (2-7%) of healthy children Both ANA and Rf may be positive in other disease conditions such as viral and bacterial infections, chronic illnesses Rf is only useful as a predictor for disease progression in Polyarticular JIA ANA should only be requested to help confirm SLE and to determine risk of uveitis in Oligoarticular JIA A positive ANA test of 1:160 in a child with musculoskeletal pain in the absence of other signs / symptoms of inflammatory or autoimmune disease is at low risk of developing such a disease Anti CCP antibodies (anti-cyclic citrullinated peptide antibody) is the most specific marker for Rheumatoid Arthritis in adults, (specificity of 98%) and has prognostic significance and value in predicting those adolescents who are likely to develop progressive arthritis Plain X-Rays are useful in excluding orthopaedic problems and malignancy but are not helpful in the diagnosis of arthritis Management: Y:\docsdata\paed\Guidelines_Paediatrics\Arthritis Jul 2014.doc Page 3 of 5

4 Any delay in treatment of arthritis puts the child at risk of ongoing joint, muscle, bone damage, gross/fine motor delay Uveitis is often asymptomatic and can occur in up to 30% of JIA Undiagnosed uveitis can cause blindness (visual loss in 50%, blindness 15-40%) Risk factors for Uveitis in JIA: o Age < 7 years o Female gender o Oligoarticular subtype o ANA positivity All patients suspected of having JIA should be referred to the paediatric rheumatology team within 6 weeks of onset of symptoms and must be seen by the team within 4 weeks of referral (BSPAR Standards of Care 2009) All patients with suspected JIA must be referred to the paediatric physiotherapists All patients with suspected JIA must be referred for uveitis screening to Mr. Kafil-Hussain All patients with suspected JIA should be started on regular NSAIDS (Ibuprofen or Naproxen) References: 1. Junilla AL et al. Chronic musculoskeletal pain in children Part 1 and 11. Am Fam Physician 2006;74: Cabral DA, Tucker LB. Malignancies in children who initially present with rheumatic complaints. J Pediatr 1999;134: Malleson PN et al. Usefulness of antinuclear antibody testing to screen for rheumatic diseases. Arch Dis Child 1997;77: Jarvis JN. Commentary- ordering lab tests for suspected rheumatic diseases. Pediatric Rheumatology 2008, Vol6:19 5. Eichenfield AH et al. Utility of Rf in the diagnosis of JRA. Pediatrics 1986 Sep;78(3): K Nishimura et al. Meta-analysis:Diagnostic Accuracy of Anti-Cyclic Citrullinated Peptide Antibody and Rheumatoid Factor for Rheumatoid Arthritis. Ann Internal Med 2007; BSPAR Standards of Care for Children and Young People with Juvenile Idiopathic Arthritis 2009 Author: Dr Gillian Baksh Consultant Paediatrician Date: July 2014 Presented to & agreed by: Paediatric Clinic Guidelines Forum on: July 2014 Ratified by: Children s Services Clinical Governance Committee on: 30thJuly 2014 Review date: July 2017 Y:\docsdata\paed\Guidelines_Paediatrics\Arthritis Jul 2014.doc Page 4 of 5

5 Joint swelling, pain or impaired function with no trauma > 4 weeks If limping in young child for < 6 wks See Limping Child Guidelines %20and%20Septic%20Arthritis%20Feb%2014a.pdf Take Full History & Examination Consider: Malignancy Acute Joint & Bone Infection Streptococcal Infection Reactive Arthritis Lyme Disease Bleeding Disorders IBD NAI SLE/ Vasculitis/JDM/ Sarcoidosis TB Hypermobility Biomechanical Pain Consider Malignancy in patient with: Pain out of proportion to clinical findings Night pain especially if asymmetrical Constitutional Symptoms Focal Tenderness on examination Rash Lymphadenopathy Hepatosplenomegaly If 6 wks also consider JIA < 6 wks Check: Blood investigations as above 6 wks and /or suspected rheumatological condition Start Regular NSAIDS: Ibuprofen 20-40mg / kg / day in 3-4 doses or Naproxen 10-20mg / kg / day in 2 doses Plain x-ray of affected joints Refer to physiotherapy bleep 5157 Treat as Appropriate If Reactive Arthritis treat with NSAIDS see BNF for dose Discuss case with Dr. Baksh (bleep 8418, ext. 2716) re: further investigation, management If SOJIA please also discuss with GOSH on ext 5334 Y:\docsdata\paed\Guidelines_Paediatrics\Arthritis Jul 2014.doc Page 5 of 5

Department of Paediatrics Clinical Guideline. Guideline for the child with possible arthritis (joint swelling/pain, loss of function)

Department of Paediatrics Clinical Guideline Guideline for the child with possible arthritis (joint swelling/pain, loss of function) Definition: Juvenile Idiopathic Arthritis (JIA) is defined as arthritis