Adolescent Rheumatology Research Articles

G46 Arrived safely? Audit of documentation of successful transfer into adult care of young people attending adolescent rheumatology clinics

Background Concerns regarding the administrative transfer of information at the paediatric and adult interface have been raised by both patients and professionals alike. Although tracking mechanisms are recommended nationally, lapse of care at time of transfer is widely reported. This includes a previous departmental audit in 2007, which revealed potential lapse of care in 25% of transferred patients. Aims Our aim was to determine the success of transfer to adult care from a paediatric rheumatology service with a coordinated transitional care programme. Methods Retrospective case note review of all transferred patients from 2007–12 at a single UK hospital. Results Data collection revealed 152 patients were transferred to 35 adult rheumatologists with 90 patients discharged to primary care. Of patients transferred, the mean age at diagnosis was 10 years (1 –16) and mean age at transfer was 17 years (16–19). 64% (n = 97) were female and 26% (n = 39) were non-caucasian. 69% (n = 105) had Juvenile idiopathic arthritis and 59% (n = 89) were on a DMARD/biologic during transfer. 37% (n = 56) had a co-morbidity. The median (mean, range) duration between last paediatric and first adult appointment was 115 days (102, 0–457). Thirteen patients were lost to follow up, including 11 with inflammatory disease, 4 with co-morbidities, and 3 on DMARD/biologic. Successful transfer, defined by receipt of a letter from first adult appointment, occurred in 86% (n = 130) of cases, and receipt of a letter from second adult appointment occurred in 43% (n = 65) of cases. Seven were currently being transferred and not included. Referral letter, health summaries, and copy letters were sent to the adult team in 76% (n = 116) of cases, and copied to 41% (n = 63) of patients. Conclusions Although there has been an improvement in transfer organisation since the previous audit, there continues to be variation within a single paediatric rheumatology service with an established transitional care programme. Further attention to the administrative workload of transition and consistency of practice within teams is required to ensure equity and quality of transitional care within centres.

A140: Pediatric Rheumatology Admissions in Chile, 2001–2010: Unavailability of a Pediatric Rheumatologist May Hinder a Correct Diagnosis

Background/Purpose:Pediatric rheumatologic diseases (PRDs) comprise a group of complex conditions that involve considerable disease burden and are sometimes life‐threatening. The American College of Rheumatology and the British Society for Pediatric and Adolescent Rheumatology recommend that a Pediatric Rheumatologist (PR) be involved in the care of children affected by PRDs. In Chile, there is a very small group of pediatric rheumatologists; most of them in Santiago. Between years 2000–2010 there were 9 centers in Santiago with a Pediatric Rheumatologist, one in Concepcion (IX Region) and one in Temuco (X Region). Patients living far from a tertiary center with a PR may miss the opportunity of a timely diagnosis and treatment. The purpose of this study was to evaluate trends in PRD admissions in Chile in a 10‐year period.Methods:We reviewed nationwide admissions due to PRDs in population younger than 18 years between 2001–2010 using national ICD‐10 coded hospital discharge databases from the Chilean Ministry of Health. Time trends of PRD admissions were assessed by linear regression. Poisson Rates Ratios were used to compare the rate of admissions for PRDs in centers with and without a PR.Results:Of 3,719,124 pediatric admissions, 15,061 (0.4%) had a discharge diagnosis of a PRD. Mean age was 7.9 ± 5 years and 53% were female. Overall lethality rate was 0.2% of admissions. While overall national admissions showed 26.3% of patients had private insurance, among pediatric rheumatology admissions private insurance accounted for 23.9%. Most prevalent diagnosis for PRD admissions corresponded to IgA Vaculitis (33%), juvenile idiopathic arthritis (16%), systemic lupus erythematosus (9%) and Kawasaki disease (8.5%). Yearly admissions due to PRDs did not have significant variations during the study period. 70% of PRD admissions occurred in hospitals with a PR. Poisson Rate Ratio comparing admissions in hospitals with and without PR revealed a significantly higher rate of PRD admissions in hospitals with a PR (PRR 1.27, 95%CI 1.23–1.31). Poisson Rate Ratios for specific PRDs showed higher rates of admission in hospitals with a PR for juvenile idiopathic arthritis (PRR 1.42, 95%CI 1.3–1.55), Kawasaki disease (PRR 2.15, 95%CI 1.87–2.47), systemic lupus erythematosus (PRR 1.63, 95%CI 1.44–1.85), and juvenile dermatomyositis (PRR 2.22, 95%CI 1.76–2.81) but not for IgA vasculitis (PRR 1.04, 95%CI 0.98–1.1).Conclusion:In Chile, hospital admissions due to a PRD are more frequent in hospitals with a PR than without. Compared with hospitals with a PR, centers without a PR appear to have similar admission rates for IgA vasculitis, suggesting non‐PRs have the ability to diagnose, treat and discharge patients with this condition, but have lower admission rates of other PRDs. These lower rates may be due to referral to tertiary centers with a PR or due to a missed diagnosis. This study reinforces the urgent need for more PRs throughout the country.

A181: Evaluating Decision‐Making in a Pediatric Rheumatology Clinic

Background/Purpose:Children with rheumatic disease often have chronic needs that require comprehensive, lifelong medical care. Transition of adolescent rheumatology patients from pediatric to adult institutions, in addition to a shift in legal responsibility, calls for the development and activation of medical decision‐making skills. Medical decision‐making is the umbrella term for the dynamic and evolving process of arriving at a medical decision, and is comprised of multiple factors including adherence, self‐management, patient activation, health consciousness, and patient empowerment. This study assessed adolescent and caregiver medical decision‐making characteristics in the outpatient Rheumatology clinic of a pediatric tertiary care hospital. We hypothesized that (1) pediatric patients who are more involved in their care have lower decisional conflict (i.e., conflict over making a medical decision), (2) adolescents want to be involved in their care, and (3) higher health consciousness is positively correlated with valuing involvement in medical decision‐making, actual involvement in decision making, and lower decisional conflict.Methods:This was an anonymous survey study of adolescent patients with chronic rheumatologic illness and their caregivers presenting to the outpatient Rheumatology clinic at Boston Children's Hospital. Both adolescent and caregiver surveys included measures pertaining to health consciousness, medical values, potential barriers to involvement in care, decisional conflict, perceived actual involvement, and desired involvement.Results:A total of twenty‐eight adolescent'caregiver pairs completed the survey. The mean age of adolescents was 16.14 years old (SD = 2.5). There was a significant positive correlation between adolescent and caregiver health consciousness (r = 0.43, p = 0.027). Valuing involvement in medical decision‐making by adolescent patients was positively correlated with their perception of actual involvement (r = 0.39, p = 0.04) and with their level of health consciousness (r = 0.48, p = 0.009). Furthermore, adolescent perception of actual involvement in medical decision‐making correlated negatively with their feelings of decisional conflict (r = −0.64, p <0.001).Conclusion:Adolescent patients with chronic rheumatic disease have considerable motivation for involvement in care. Given the link between treatment adherence and medical decision‐making, the characteristics of medical decision‐making in the pediatric rheumatology population are a valuable area for future research.

The coming of age of adolescent rheumatology

The goal of planned adolescent health-care transition procedures is to optimize functioning and well-being for all young people, including those who have special health-care needs. In this regard, the transitioning of young people with childhood-onset rheumatic diseases to adult health care is increasingly important, particularly as many of these patients might continue to have active disease or considerable sequelae well into their adult lives. Key components of a successful plan for health-care transition include encouragement of patient self-advocacy, tailoring of the process to each individuals' needs, family adaptation, and readiness and training of relevant health-care providers. Improving outcomes in patients with serious rheumatic diseases presenting in adolescence will be achieved by increasing our understanding of the aetiopathogenesis of these disorders, identifying accurate predictors of the development and/or course of disease and better defining long-term outcomes. In this article, we discuss transitional health-care models, as well as the benefits and challenges of providing transitional care to adolescents with rheumatic diseases. We also highlight the need to ensure that research is integral to transitional care pathways.

PReS-FINAL-2249: A national survey of the role of the paediatric rheumatology nurse in performing steroid joint injections in the UK

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. Treatments involve Non Steroidal Anti-Inflammatory Drugs (NSAIDs), Disease Modifying Anti-Rheumatic Agents (DMARDs), steroids and various biologic drugs. Steroid injections directly into affected joints are an important part of the range of treatments available for children with JIA. The British Society of Paediatric and Adolescent Rheumatology (BSPAR) guidelines state that all patients with JIA will have access to intra-articular joint injections as required, with access to entonox, general anaesthesia and appropriate imaging technology where necessary. To meet the increasing demand for steroid joint injections, Rheumatology nurses and other rheumatology allied health professionals have started training to carry out this extended role in some parts of the UK.

PReS-FINAL-2072: How does the management of enthesitis related arthritis differ from other sub-types of juvenile idiopathic arthritis? A retrospective study of jia at an adolescent rheumatology centre

Enthesitis Related Arthritis (ERA) is a sub-type of JIA with emerging phenotypic differences. Clinical onset is often insidious and there is evidence to suggest long-term remission is less common. There is limited data comparing the treatment of ERA to other sub-types for the adolescent age spectrum.

Methotrexate-induced nausea and vomiting in adolescent and young adult patients

This study aims to determine the prevalence of methotrexate-induced nausea and vomiting in both adolescent and adult patients with inflammatory arthritis. A survey of methotrexate side effects was conducted on patients with inflammatory arthritis. We provided a brief questionnaire to unselected patients with inflammatory arthritis being treated with methotrexate attending adolescent and adult rheumatology clinics. The questions related to the presence, absence, and severity of nausea and vomiting, the temporal relationship with methotrexate and whether anti-emetics had been prescribed. A total of 106 patients from the age of 13 years and above—57 adults (over 20 years) and 49 adolescents (13–19 years) were included in this study. The median age for those experiencing nausea was 19 years (interquartile range (IQR) 7) and for those with no nausea 55 years (IQR 46) (p < 0.001). Thirty-six out of 49 adolescent patients reported nausea (73 %) compared to only 20/57 adults (35 %) (p < 0.001). Multiple logistic regression analysis showed that the nausea group had a significantly higher proportion of adolescents (p = 0.0002), patients taking subcutaneous (SC) methotrexate MTX (p = 0.002), and patients with duration of MTX of more than 1 year (p = 0.049). Adolescents were estimated to have over 6 times higher odds of nausea compared to adults (OR 6.31, 95 % CI 2.38 to 16.75, p = 0.0002) after adjusting for SC MTX and duration of MTX. Only 22 % of adolescents and 10 % of adults were prescribed anti-emetics. There is a higher prevalence of MTX-induced nausea and vomiting in adolescents and younger adult patients with inflammatory arthritis compared to older adults. The role of anti-emetics in the treatment of these symptoms is unclear.

SAT0450 Treatment with Leflunomide Results in a Higher Flare Rate of Chronic Uveitis Compared to Methotrexate in Patients with Juvenile Idiopathic Arthritis Treated with Both Drugs

BackgroundChronic anterior uveitis is a common complication of juvenile idiopathic arthritis (JIA). Leflunomide is a frequently used alternative to methotrexate in the treatment of joint manifestations of JIA. However, very...

SAT0453 Have We Got the Eyes Right? An Audit of Referral Patterns to Opthalmology in JIA

BackgroundUveitis is a complication of Juvenile Idiopathic Arthitis (JIA) and can lead to chronic visual impairment. Each type of JIA has a different prevalence. Oligoarticular onset JIA has a high...

AB0019 A genetic association between juvenile idiopathic arthritis and the il10 polymorphism

BackgroundJuvenile idiopathic arthritis (JIA) is a generic term for arthritis that has an onset before the age of 16 and persists for more than 6 weeks. JIA is probably a...

AB1116 Selective ig a deficiency in juvenile SLE patients

BackgroundImmunoglobulin A (IgA) is responsible for protection against infections of the respiratory and gastrointestinal tracts, and selective IgA deficiency (SIgAD) is the most common humoral immunodeficiency. It is speculated that...

Physical and social functioning in adolescents with rheumatological conditions: a study of predictors

This study examines possible predictors of physical and social functioning in adolescents with rheumatological conditions. Condition-related variables and psychosocial variables were studied, and their relative contribution as predictors was examined. The study population was one hundred and twelve adolescents (11-18years) attending secondary and tertiary paediatric rheumatology outpatient clinics in south-west England. These adolescents completed validated self-report accounts of disease history, pain and functioning (condition-related variables) and the Bath Adolescent Pain Questionnaire (psychosocial variables). Correlation and regression analyses were used to establish influences on physical and social functioning, examining condition-related variables and psychosocial variables as separate blocks. Physical functioning was independently associated with age at onset, intensity of pain, presence of depression and pain-specific anxiety. Social functioning was only associated with general anxiety. The presence of an inflammatory diagnosis had no bearing on optimal functioning in this study. Condition-related variables (age at onset, pain intensity) and psychosocial variables (depression, pain-specific anxiety) were equally important for physical functioning, whereas psychosocial variables (general anxiety) were more influential for social functioning. Understanding the impact of disease and associated variables in the adolescent rheumatology population should optimize targeted multidisciplinary rehabilitation for the young person and their family.

The MAGICC and practical approach to rheumatology transition

This article describes the inception and continuing evolution of a seamless adolescent rheumatology service across a children's and adult hospital. This service is still a work in progress but its principles are embedded in the MAGICC approach: Moving on in adolescence, Growing up In Collaboration and Coping.

Matrix Disruptions, Growth, and Degradation of Cartilage with Impaired Sulfation

Diastrophic dysplasia (DTD) is an incurable recessive chondrodysplasia caused by mutations in the SLC26A2 transporter responsible for sulfate uptake by chondrocytes. The mutations cause undersulfation of glycosaminoglycans in cartilage. Studies of dtd mice with a knock-in Slc26a2 mutation showed an unusual progression of the disorder: net undersulfation is mild and normalizing with age, but the articular cartilage degrades with age and bones develop abnormally. To understand underlying mechanisms, we studied newborn dtd mice. We developed, verified and used high-definition infrared hyperspectral imaging of cartilage sections at physiological conditions, to quantify collagen and its orientation, noncollagenous proteins, and chondroitin chains, and their sulfation with 6-μm spatial resolution and without labeling. We found that chondroitin sulfation across the proximal femur cartilage varied dramatically in dtd, but not in the wild type. Corresponding undersulfation of dtd was mild in most regions, but strong in narrow articular and growth plate regions crucial for bone development. This undersulfation correlated with the chondroitin synthesis rate measured via radioactive sulfate incorporation, explaining the sulfation normalization with age. Collagen orientation was reduced, and the reduction correlated with chondroitin undersulfation. Such disorientation involved the layer of collagen covering the articular surface and protecting cartilage from degradation. Malformation of this layer may contribute to the degradation progression with age and to collagen and proteoglycan depletion from the articular region, which we observed in mice already at birth. The results provide clues to in vivo sulfation, DTD treatment, and cartilage growth.

Evidence-based, interdisciplinary guidelines for anti-inflammatory treatment of uveitis associated with juvenile idiopathic arthritis

Uveitis in juvenile idiopathic arthritis (JIA) is frequently associated with the development of complications and visual loss. Topical corticosteroids are the first-choice therapy, and immunosuppression is commonly used. However, treatment has not been standardized. Representatives from the German Ophthalmological Society, Society for Childhood and Adolescent Rheumatology, and the German Society for Rheumatology reached consensus on a standardized treatment strategy according to disease severity in the individual patient. The recommendations were based on a systematic literature analysis in MEDLINE and consensus expert meetings. Evidence and recommendations were graded, and an algorithm for anti-inflammatory treatment and final statements confirmed in a Delphi method. An interdisciplinary, evidence-based treatment guideline for JIA uveitis is presented.

Auto-antibody testing for children on biologic therapies for rheumatological conditions: results of audit

Background Biologic agents may cause auto-antibody formation and drug induced lupus but no paediatric guidelines exist regarding monitoring of auto-antibodies. The UK Royal College of Nursing Guidance advises checking ANA and anti-dsDNA before starting biologic treatment and repeating anti-dsDNA if concerned. The Alder Hey protocol tested an autoantibody profile (Table 1, 2) 3-6 monthly for data entry for the British Paediatric and Adolescent Rheumatology (BSPAR) Biologics and New Drugs Registry (BDNR) and children with day ward IV biologic administration often had automatic antibodies testing 4-6 weekly.

Varicella immunity and exposure in JIA patients on methotrexate therapy

AimsMethotrexate has been used in Juvenile Idiopathic arthritis (JIA) for 80 years and is a first line agent, despite the proliferation of newer therapies. British Society of Paediatric and Adolescent...

Duration of etanercept treatment and reasons for discontinuation in a cohort of juvenile idiopathic arthritis patients

Since 2004, juvenile idiopathic arthritis (JIA) patients treated with etanercept and/or MTX have been monitored in the British Society for Paediatric and Adolescent Rheumatology Biologics and New Drug Register. Here, we report the duration of etanercept use for the first 5 years of the register and reasons for discontinuation. Disease subtype and activity, comorbidity, treatment efficacy and safety data were recorded. Etanercept discontinuation was defined as stopping the drug because of disease remission or treatment failure. Time to discontinuation was explored using Kaplan-Meier survival analysis with remaining patients censored at 5-year follow-up. A total of 483 etanercept-treated JIA patients were enrolled from 30 UK centres, representing 941 patient-years of follow-up. A total of 100 (20.7%) patients discontinued etanercept; 9 due to disease control, 88 because of treatment failure, 2 for unknown reasons and 1 because of a change in diagnosis. Of the 53 patients in whom etanercept was perceived to be ineffective at controlling the inflammation, 48 were prescribed other biologic drugs [26/48 (54%) infliximab]. In 21 patients with intolerance, infections, CNS events and a few isolated events were associated with discontinuation. Using Kaplan-Meier analysis, at 5 years 69% (95% CI 61, 77%) had not experienced treatment failure. Discontinuation of etanercept for inefficacy was associated with systemic arthritis subtype [odds ratio (OR) 2.55, 95% CI 1.27, 5.14], chronic anterior uveitis (OR 2.39, 95% CI 1.06, 5.35) and inefficacy of MTX before starting etanercept (OR 8.3, 95% CI 1.14, 60.58). In a cohort of JIA patients treated with etanercept and followed for a median of 2 years (maximum 5 years), the majority (69%) remain on the drug.

British Isles Survey of Methotrexate Monitoring Practice During Treatment of Juvenile Idiopathic Arthritis

We surveyed current pediatric rheumatology monitoring practice in methotrexate treatment of juvenile idiopathic arthritis in the British Isles, and experiences of significant side effects during methotrexate monitoring. Single-center responses were sought from the current British Society for Pediatric and Adolescent Rheumatology membership, using a web-based survey tool. Thirty-three centers across the British Isles responded. Twenty-eight centers reported following British Society for Pediatric and Adolescent Rheumatology or local guidelines. Thirty-one centers were willing to modify their monitoring practice to individual circumstances. All centers used a full blood count and liver enzymes as monitoring tests. There was variation in frequency at which monitoring tests were performed, both at initiation of methotrexate therapy and once methotrexate therapy was established; 27 centers were willing to change the frequency of monitoring blood tests after a period of stability. Centers reported acting on alanine aminotransferase values ranging from 50 to 500 IU/L. Particular variation existed between smaller and larger centers. Few centers reported any experience of serious side effects, and only 1 cited a case of liver cirrhosis. Despite specific pediatric guidance for monitoring low-dose methotrexate use in juvenile idiopathic arthritis, variation in practice exists in the British Isles. It may be that blood test monitoring could safely be performed less frequently than currently recommended. To inform future changes to guidance, we suggest establishing a prospective registry for serious side effects.

Standards of care for JIA—the basic foundation for quality

In terms of quality of healthcare, standards of care establish the basic principles and infrastructure required to achieve optimal health outcomes for a specific patient population. In the UK, the British Society for Pediatric and Adolescent Rheumatology (BSPAR) standards of care for juvenile idiopathic arthritis (JIA) are an excellent example of this foundational work.