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H. Ali, M. Friswell, T. Bell, H. E. Foster. Departments of Rheumatology and Child Health, University of Newcastle, Newcastle upon Tyne Hospitals NHS Trust

Introduction: There is an increasing trend to earlier and aggressive treatment in juvenile idiopathic arthritis (JIA), with emerging evidence that earlier intervention is associated with better outcome. Owing to long waiting times for routine new patient clinics, a fast track clinic was established in September 2002 to prioritise children with inflammatory arthritis and facilitate early assessment and management by the multidisciplinary team (MDT).

Aim: To assess the accuracy of triaging referral letters by clinician to either fast track or routine clinic appointment of new patients.

Method: 91 consecutive new referral letters were analysed along with new patient clinic letters. Two consultants (MF and HF) independently categorised referral letters as JIA/inflammatory or non-inflammatory. All children have been reviewed by both consultants in the clinics. Triage diagnosis v final clinic diagnosis was compared to measure sensitivities, specificities, and predictive values.

Results: 54 females and 37 males with a median age of 10.7 years. 54 (59.3%) of referrals were made by GPs, the remaining either by paediatricians or orthopaedics surgeons. 18 cases had inflammatory arthritis (16/18 (88.9%) JIA, SLE n = 1, Down’s associated arthritis n = 1). The remaining 73/91 had non-inflammatory conditions. Between the consultants, for triage and final diagnosis, the sensitivity for detecting inflammatory arthritis was 100% each with specificities of 68.49% and 60.27%, positive predictive values of 44% and 38%. Current waiting time is 4 weeks for fast track and 9 weeks for routine new patient appointments.

Conclusion: Both consultants were able to reliably triage referral letters, as no case with inflammatory arthritis was missed. However, improving triage specificity is required for optimum utilisation of the fast track clinics.


K. L. Shaw, T. R. Southwood, J. E. McDonagh, the British Paediatric Rheumatology Group. Children’s Chronic Arthritis Association, Lady Hoare Trust & Arthritis Care; Institute of Child Health, University of Birmingham, Birmingham B15 2TT, UK

Background: This study reports the disease knowledge of young people with JIA and their caregivers with specific reference to the name of their disease.

Methods: A knowledge questionnaire was administered to 308 adolescents with JIA and 301 of their caregivers and included the following two questions: 1) what does JIA stand for?; and 2) when you become an adult, what will your type of arthritis be known as? The response format included six multiple choice answers including ‘don’t know’.

Results: The median age of adolescents was 14 (11–18) years and median disease duration was 6 (0–16) years.

Further analyses showed that there were no significant differences in sex, age, or age at onset between those who answered correctly and those who did not.

Conclusions: Many young people with JIA and their caregivers cannot correctly identify their disease, which has implications for future disclosure to others and information seeking behaviours.

Abstract G125


M. S. Eltringham1, A. Myers2, S. J. Proctor1, P. Taylor1, L. Parker1, M. Abinun2, M. Friswell2, L. J. Kay2, H. E. Foster1. 1School of Clinical Medical Sciences, University of Newcastle-upon-Tyne, UK; 2Newcastle-upon-Tyne Hospitals NHS Trust, UK

Aim: The trend to earlier and more aggressive treatment for JIA is based on emerging evidence that this improves outcome. It is recommended that all patients be managed by a paediatric rheumatology multidisciplinary team (MDT). This study prospectively documents pathways of care and management in JIA to determine the impact of process of care on outcome.

Methods: Details for consecutive new patients with JIA have been collated (clinical presentation, referral pathways, investigations, management, and outcome).

Results: 71 children with JIA have been recruited (oligo onset (n 33), extended oligo (6), polyRF− (13), polyRF+ (2), systemic (7), psoriatic (3), and other (7)). Most (94%) children had a diagnosis of JIA made at the first paediatric rheumatology assessment, with a median age of 8 years (1–17) and median interval from symptom onset being 20 weeks (0–416). At presentation to paediatric rheumatology, many patients had functional disability (CHAQ score >0 (52%), active joint disease (70%), >1 restricted joint (46%)). Investigations prior to paediatric rheumatology included radiographs (22), ultrasound (7), MRI (5), bone scan (3), joint aspiration (7) (+ injection (2)), and arthroscopy and biopsy (5). Only 6/71 had been assessed by physio/OT, and only 6/71 had their eyes screened for uveitis. Most were not on regular medication (NSAIDS (21), methotrexate (3)). Following paediatric rheumatology first assessment, however, all were assessed by the MDT within 6 weeks (72% <2 weeks) and screened for uveitis, 43 (61%) patients had joint injection(s), 13 (18%) were started on methotrexate, and 16 (23%) on corticosteroids (5/16 pulse IV steroids, 11/16 oral steroids).

Conclusions: Delay to diagnosis and starting recommended management is common in incident cases of JIA. Many children at initial paediatric rheumatology assessment have marked functional disability, untreated active disease, and have not been screened for uveitis. The duration of untreated disease is likely to adversely affect response to treatment and outcome. The needs of children presenting with JIA are not being met.


K. L. Shaw, T. R. Southwood, J. E. McDonagh. the BPRG, CCAA, LHT, and AC. Institute of Child Health, University of Birmingham, Birmingham, B4 6NH, UK

Background: The purpose of the present study was to explore best practice and feasibility in the transitional care of adolescents with JIA from the perspectives of multidisciplinary rheumatology providers and service users.

Methods: A modified two round Delphi study was undertaken with rheumatology health providers, young people with JIA (aged 12–25 years) and their parents. Participants were asked to rate the extent to which 26 statements about transitional care constituted best practice. Providers were also asked to rate their feasibility.

Results: Second round questionnaires were completed by 74 (90%) individuals. The providers had worked in rheumatology for a mean of 11.5 (range 1.5–11.5) years and saw 17.0 (range 0.0–70.0) adolescents per month. Young people had a mean age of 16.4 (range 12.0–21.1) years, disease duration of 11.8 (3.0–20.0) years. Items strongly agreed to constitute best practice and highly feasible included addressing young people’s psychosocial and educational/vocational needs; using an individualised approach; providing honest explanations of the adolescents condition and healthcare; providing opportunities for adolescents to express opinions and make informed decisions; having continuity in health personnel; and giving adolescents the option of being seen by professionals without their parents. Providing adolescent focused environments, professionals knowledgeable in transitional care and opportunities to meet similar others were seen as feasible in only a few hospitals.

Conclusions: Those aspects of transitional care that are easily achievable should be undertaken in all hospitals that care for adolescents with JIA. Future research needs to target those aspects that are deemed as important, but not feasible, to determine how these needs can be met.


P. J. C. Davis, J. E. McDonagh, C. A. J. Ryder, T. R. Southwood. Department of Paediatric Rheumatology, Birmingham Children’s Hospital, UK

Aim: Only two published studies detail the outcome of JIA patients after methotrexate was stopped, but neither used a prolonged complete clinical response (PCCR) of at least 1 year as a criterion for stopping. Our aim was to compare the outcome and clinical features of 29 patients who stopped methotrexate after PCCR with 22 patients who stopped methotrexate for lack of efficacy (LOE: 8) or intolerance (Intol: 14).

Methods: We used a descriptive, retrospective casenote review with standardised data recording. Most patients were classified as either polyarthritis (PA: 31) or systemic arthritis (SA: 9). The rest (other) comprised oligoarthritis (3), psoriatic (6), and enthesitis related (2).

Results: See tables.

Abstract G128, table 1

Abstract G128, table 2 Outcome of PCCR patients

Conclusions: Kruskal-Wallis test showed no difference in age at disease onset or duration of methotrexate treatment for patients who stopped methotrexate due to PCCR compared with those who stopped due to LOE or Intol. A greater proportion of PCCR patients with SA (4/5) than PA (8/21) remained in remission after methotrexate was stopped. 2-tailed t test showed no significant difference in duration of PCCR between patients who remained in remission and those who relapsed.


S. Deeley, J. Gondwe, J. A. Sills, J. E. Davidson, A. G. Cleary. Royal Liverpool Children’s NHS Trust, Department of Rheumatology, Eaton Road, West Derby, Liverpool, L12 2AB

Aim: The withdrawal from manufacture of triamcinolone hexacetonide (TH) has led to an increased use of triamcinolone acetonide (TA) for IA injection in JIA. This study aims to compare the efficacy and adverse effects of these preparations.

Method: A retrospective review of case sheets of children who received an IA injection between March 2000 and April 2003. Patient demographics were collected, including age, sex, JIA subtype, and concomitant treatment with disease modifying drugs. The joint(s) injected, steroid preparation and dosage, and the child’s weight were determined. A positive clinical response was defined as no or minimal signs of arthritis in the injected joint at the next assessment. Time to repeat injection and any adverse events were recorded.

Results: 115 children received 376 injections; 256 receiving TH, 113 receiving TA, and 7 used an alternative steroid preparation. Follow up data were collected on 237 receiving TH and 96 receiving TA. The commonest joints injected were knees and ankles (288/376). The groups receiving TH and TA appeared similar for patient demographics including disease subtype. The mean dose of TH and TA for large joints (hip, knee, and ankle) was 0.9mg/kg and 1.6mg/kg, respectively. The overall remission rate for TH was 83.12% and 87.5% for TA. 75/237 (29%) of TH injected joints required re-injection, and 25/96 (26%) for TA. The median time to re-injection was 8.7 months for TH and 6.16 months for TA. A self limiting but distressing cushingoid state was noted in 5 patients receiving TA (median number of joints injected 6, range 1-8), none after receiving TH. No other significant adverse effects were noted.

Conclusion: The efficacy of TH and TA appears broadly similar in this retrospective study. The higher solubility of TA may account for the increased effect of cushingoid state post injection. Because of the retrospective nature of this study the results need to be interpreted with caution.


K. Khawaja, M. Abinun, M. Friswell, H. Foster. Department of Paediatric Rheumatology, Newcastle upon Tyne NHS Trust, Newcastle, Tyne and Wear

Aim: Long term steroids have a wide range of undesirable side effects and assessment of these varies widely. We wished to examine how children are currently monitored and to use this to propose guidelines for future use.

Methods: A literature review was undertaken, which found no national guidelines for children on long term steroids. A questionnaire was then sent to all 40 paediatricians in the trust, asking about individual practice for paediatric patients either on, or about to commence, long term steroid therapy. We looked at osteoporosis, varicella contacts and infection, immunisations, and routine monitoring and follow up measures. 20 replies were received including all different specialities across the directorate.

Results:Osteoporosis: nine gave advice on osteoporosis, (four gave advice on weight bearing exercise, seven on smoking/alcohol). Three prescribed routine calcium supplements; five agreed that dose of steroids influenced their decision to give calcium supplements; and two did not usually prescribe them (two also felt the duration of steroid use influenced the decision to give calcium supplements). 10 referred all patients on steroids for DEXA scans, 10 had criteria for referral (nine with history of low trauma fracture, two with x ray changes of osteopaenia, one with duration of therapy >6 months, and one with duration of therapy >12 months). Varicella: four had a formal policy for chicken pox contact while on steroids, and a further 16 described their personal practice. Seven checked varicella immune status in patients suspected to be on long term steroid therapy. Immunisation: no consistent policy was being followed. Monitoring: 10 regularly monitored patients on long term steroids. Six gave an information leaflet and only two gave steroid alert card.

Conclusions: From this survey we conclude that the care of children on chronic steroids varies widely. We prepared consensus guidelines for all the aspects covered in this survey and they are currently in use and available for comment.


E. J. Lim, J. Ferrari, H. Hasson, K. Murray. Department of Paediatrics, Ealing Hospital, London; Department of Rheumatology, Institute of Child Health, London

Aim: There are currently no specific clinical tools to assess hypermobility in children. Our aim was to develop modified criteria and a scoring system for the assessment of potentially clinically relevant hypermobility in children and to compare the modified score with the current standard used in adults (the Beighton score).

Method: 305 primary school children were examined using a series of passive joint manoeuvres. The modified score assessed 19 manoeuvres in 18 joints compared with the Beighton score, which assessed 9 manoeuvres in 9 joints.

Results: The modified score identified 8% of the sample population as hypermobile. The standard Beighton score identified 18% as hypermobile. The modified score was significantly (p<0.002) more accurate at diagnosing hypermobility, with a positive predictive value of 80% and specificity of 98%. Examiners agreed on the diagnosis 80% of the time and intraobserver variability was 90%.

Conclusions: The modified criteria and score are more sensitive and specific than the Beighton score. The high specificity allows a positive diagnosis to be made, therefore, making it a useful tool to identify hypermobility in children.


R. Lamb, E. Zeggini, W. Thomson, BPRG. study group, R. Donn. ARC, University of Manchester

Background: JIA is the commonest chronic rheumatic disease of childhood. Progressive pseudorheumatoid dysplasia (PPD) is a rare, severe, skeletal disorder, which clinically mimics JIA but is non-inflammatory. Several distinct mutations of the WISP3 gene have previously been shown to be causal of PPD. WISP3 is therefore an important candidate gene for investigation in JIA.

Aims: To identify polymorphisms of WISP3 and determine their role in JIA susceptibility.

Patients and Methods: Mutation screening, by dHPLC, of five exons of theWISP3 gene was carried out in a cohort of 86 UK Caucasian JIA, 35 non-Caucasian JIA (all with >5 affected joints), and 20 controls. Genotyping of the identified SNPs was then performed in a cohort of UK Caucasian JIA (all with >5 affected joints) (n = 164) and unrelated controls (n = 256), using Taqman allelic discrimination (3700, Applied Biosystems).

Statistical Methods: Comparison of genotype, allele, and haplotype frequencies was by the χ2 test. Haplotypes were inferred using the EM algorithm.

Results: No JIA specific WISP3 SNPs were identified. Three SNPs were observed, WISP3+99 (A to C), WISP3+381 (C to T), and WISP3+822 (A to C) (Embl: AF100781). No deviation from HW equilibrium was found in the JIA cases or the controls. No association was found for any of the SNPs or haplotypes with JIA susceptibility.

Discussion: This is the first investigation of the WISP3 gene in JIA. The polymorphisms found from the exonic mutation screening we have carried out do not appear to increase the risk of JIA susceptibility.

Abstract G132 Allele frequencies 2n (%) of WISP3 SNPs in JIA cases and controls


P. Riley, C. Pilkington. Juvenile Dermatomyositis Research Centre, Institute of Child Health and Great Ormond Street, London

Aims: To determine the incidence of secondary hyperlipidaemia in JDM and its relation to disease activity and treatment.

Methods: Non-fasting lipid profile was assessed in all JDM patients attending the ward and clinic over a 6 month period. Inflammatory markers, muscle enzymes, and clinical parameters were recorded.

Results: 38 patients had 1–3 profiles performed. 11 of these patients had hyperlipidaemia. All 11 had hypertriglyceridaemia and three also had hypercholesteraemia. Of the 11, five had low high density lipoprotein (HDL), three normal HDL, and one increased HDL; and in two HDL was not performed. Of the 11 patients, three were new presentations, seven had chronic JDM activity, and one was well but also a type one diabetic. The three new presentations were untreated. Two of the chronic patients were not on prednisolone, and five chronic patients had received steroids in the previous 6 months. Of the 11 patients only six had ESR >20 and only one had CRP raised.

Conclusions: It is known that raised cholesterol, triglycerides, and decreased HDL are independently related to increased risk of atherosclerosis. A similar lipid profile is seen in SLE patients who have been shown to have premature atherosclerosis. Secondary hyperlipidaemia in our population was related to disease activity. Chronic disease activity may be linked with prolonged periods of hyperlipidaemia. The effect of this on the vascular health of the patients is unclear, but increased risk of premature atherosclerosis is a theoretical concern in JDM. Further studies, both epidemiological and those directly examining vascular physiology, are indicated.


K. Nistala1, C. Owens2, R. de Bruyn2, C. Pilkington1. Departments of Paediatric Rheumatology1 and Radiology2, Great Ormond Street Hospital, London, UK

Background: Optimum treatment in JDM requires an accurate, validated assessment tool to identify remission or relapse. Current methods used for assessing disease activity have limitations. Our aim was to test if muscle ultrasound could offer a responsive method for assessing disease activity in JDM.

Methods: We used grey scale ultrasound to prospectively scan thigh muscles in 17 children with JDM (mean age 11.3 years (4.8–14.8), mean disease duration 3.6 years (0.2–9.3)). Ten patients had a repeat scan (mean interval 80 days (29 to 104)). Images were scored by two radiologists blinded to clinical details, using a 1–4 point ordinal scoring system (1 = normal, 4 = very high echogenicity). The results were correlated with a clinical measure of muscle strength (CMAS).

Results: Muscle strength (median CMAS 50, (8–53)) had a significant negative correlation with echogenicity (Spearman’s rank −0.72, p <0.05, median echogenicity 2 (range 1–4)). Serial improvements in strength were associated with a reduction in echogenicity (Spearman’s rank −0.72, p <0.05). Our scoring system showed moderate interobserver variability for the anterior and lateral thigh (κ score up to 0.59). Scans of the medial thigh were often heterogeneous, had poor κ scores (0.27), and were excluded from the final analysis.

Conclusions: Our data support the use of muscle ultrasound for assessing disease activity in JDM. Ultrasound images taken on a single occasion correlated well with muscle weakness. Our initial findings show that serial improvements in strength, with disease remission, were mirrored by reduced muscle echogenicity. Further studies are needed to assess the sensitivity of ultrasound in detecting the relapse of myositis and whether ultrasound can be used serially as a pragmatic means of monitoring JDM.

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