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Objectives The primary objective was to define the incidence of JSLE in children <16 years of age in the UK and Republic of Ireland (ROI). The secondary objective was to describe presenting features, classification criteria, initial management and disease damage in newly presenting JSLE patients. Methods A prospective JSLE epidemiological study was undertaken between September 2017 and September 2019 with support of the British Paediatric Surveillance Unit and other professional groups involved in diagnosis and management of JSLE patients. Treating consultants reported all cases of JSLE seen. A follow-up study at 1 year examined management and progression of disease and treatment. Results There were 124 incident cases included in the final analysis. Incidence was estimated using ACR-1997 classification criteria (0.36/100 000), SLICC-2012 classification criteria (0.41/100 000) and clinician expert opinion (0.46/100 000). A high disease burden was seen, with 71.0% of patients requiring ongoing systemic CS treatment at 1 year; 98.2% receiving immunomodulatory treatment; and 20.4% accruing damage in the year following diagnosis (predominantly neuropsychiatric-related), with substantial involvement from multiple speciality teams. Conclusions The minimum UK and ROI incidence of JSLE is between 0.36 and 0.46/100 000, depending on the case definition used. Challenges in classification of patients with JSLE are highlighted, but overall this study supports the use of SLICC-2012 classification criteria. The high levels of disease damage and ongoing CS use 1 year after diagnosis is concerning, highlighting the need for further interventions to improve outcomes in JSLE. [ABSTRACT FROM AUTHOR]

Objectives To compare the treatment approaches and disease outcomes of children with JDM followed in two European tertiary care peadiatric rheumatology centres. Methods The medical notes of patients with JDM seen at Istituto Giannina Gaslini (IGG) of Genoa, Italy or Great Ormond Street Hospital (GOSH) of London, UK between January 2000 and December 2015 within 6 months after disease onset and followed for at least 6 months were reviewed. Demographic, clinical and therapeutic data were collected. At each visit, the caring physician was asked to rate the disease state subjectively. Results A total of 127 patients were included, 88 at GOSH and 39 at IGG. At 24 months, the median values of muscle strength and disease activity were at the normal end of the scale and around three quarters of patients were said to have inactive disease. Also, at 2 years, 38.6% and 36% of British and Italian patients, respectively, had damage. Cyclophosphamide, azathioprine, infliximab, rituximab and mycophenolate mofetil were used more frequently by UK physicians, whereas ciclosporin, intravenous immunoglobulin and hydroxychloroquine were prescribed by Italian physicians. Conclusion This study shows a significant difference in the choice of medications between pediatric rheumatologists practising in the two centres. Despite this, a high proportion of patients had inactive disease at 2 years and there was a low frequency of damage: modern treatments have improved outcomes. [ABSTRACT FROM AUTHOR]

The article discusses research which investigated the clinical and laboratory characteristics of monogenic autoinflammatory disorders that mimic Behçet's disease (BD) in children. Topics explored include the challenges associated with the diagnosis of BD, the BD-related clinical manifestations observed in pediatric subjects, and the necessity of genetic screening of paediatric BD patients.

Objectives. The medical management of JIA has advanced significantly over the past 10 years. It is not known whether these changes have impacted on outcomes. The aim of this analysis was to identify and describe trends in referral times, treatment times and 1-year outcomes over a 10-year period among children with JIA enrolled in the Childhood Arthritis Prospective Study. Methods. The Childhood Arthritis Prospective Study is a prospective inception cohort of children with new-onset inflammatory arthritis. Analysis included all children recruited in 2001-11 with at least 1 year of follow-up, divided into four groups by year of diagnosis. Median referral time, baseline disease pattern (oligoarticular, polyarticular or systemic onset) and time to first definitive treatment were compared between groups. Where possible, clinical juvenile arthritis disease activity score (cJADAS) cut-offs were applied at 1 year. Results. One thousand and sixty-six children were included in the analysis. The median time from symptom onset and referral to first paediatric rheumatology appointment (22.7-24.7 and 3.4-4.7 weeks, respectively) did not vary significantly (~20% seen within 10 weeks of onset and ~50% within 4 weeks of referral). For oligoarticular and polyarticular disease, 33.8-47 and 25.4-34.9%, respectively, achieved inactive disease by 1 year, with ~30% in high disease activity at 1 year. A positive trend towards earlier definitive treatment reached significance in oligoarticular and polyarticular pattern disease. Conclusion. Children with new-onset JIA have a persistent delay in access to paediatric rheumatology care, with one-third in high disease activity at 1 year and no significant improvement over the past 10 years. Contributing factors may include service pressures and poor awareness. Further research is necessary to gain a better understanding and improve important clinical outcomes. [ABSTRACT FROM AUTHOR]

Objectives. The objectives of this study were to investigate change in disease activity, and explore factors associated with response, in children with JIA over the initial year of etanercept treatment. Methods. This analysis included children with JIA starting etanercept in the British Society for Paediatric and Adolescent Rheumatology Etanercept Cohort Study. Response was assessed using change in juvenile arthritis disease activity score-71 (JADAS-71), an excellent response (ACR Pedi 90), and achieving minimal disease activity (MDA) at 1 year. Change in JADAS-71 was evaluated over time. Multivariable backward stepwise logistic regression was performed to identify factors associated with ACR Pedi 90 and MDA. Results. A total of 496 children were included. Over the first year, 17 stopped due to inefficacy, 9 due to adverse events and 7 for other reasons. One child stopped for remission. At 1 year, 74, 69 and 38% reached ACR Pedi 30, 50 and 90, respectively, and 48% had achieved MDA. Independent predictors of achieving ACR Pedi 90 at 1 year included shorter disease duration [odds ratio (OR) 0.91; 95% CI: 0.85, 0.97)], no concurrent oral corticosteroid use (OR 0.48; 95% CI: 0.29, 0.80) and history of uveitis (OR 2.26; 95% CI: 1.08, 4.71). Independent predictors of achieving MDA at 1 year included younger patients (OR 0.60; 95% CI: 0.38, 0.95), and disease not treated with concurrent oral corticosteroids (OR 0.57; 95% CI: 0.35, 0.93). Conclusion. Among this real-world cohort of children with severe JIA, a significant proportion of children achieved an excellent ACR Pedi response and MDA within 1 year of starting etanercept, although few clinical factors could predict this outcome. [ABSTRACT FROM AUTHOR]

Background Rituximab is an anti-CD20 chimeric monoclonal antibody that depletes pre-plasma B cells. It is used in paediatric rheumatology for conditions including juvenile systemic lupus erythematosus (jSLE), juvenile dermatomyositis (JDM), vasculitides and refractory juvenile idiopathic arthritis (JIA). We aim to describe the cohort of patients treated with rituximab in the Scottish Paediatric and Adolescent Rheumatology Network (SPARN) in a 5-year period, and assess the need for a network guideline. Methods A review of paper and electronic case records. Patients seen within the SPARN network who received Rituximab between 2014 and 2018 were included. Results Vasculitis Rituximab was given to 16 patients in 5 different SPARN centres between 2014 and 2018. Male: Female ratio was 1:4.3. Mean age at first dose of rituximab was 12.75 years. Mean disease duration pre rituximab was 1 year. 8 (50%) patients had jSLE, 7 multisystem and 1 isolated lupus nephritis. 3 had ANCA-positive vasculitis and 5 had other conditions. (1 had RF+ve JIA; 1 severe panuveitis; 1 had an unspecified autoinflammatory condition; 1 had COPA syndrome with severe interstitial lung disease and 1 had autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). Documentation of pre-rituximab checks was poor. Baseline bloods were done in all 16 patients. 11 patients had lymphocyte subsets and 13 immunoglobulins documented. Disease-specific antibodies were measured in all patients in whom applicable (11). 11 had a chest x-ray prior to rituximab. In 9 cases a pre-rituximab pregnancy test was indicated: none were documented. In only 3 patients was a pre-rituximab history of recent infections documented. In 8 patients a discussion regarding treatment risks and benefits was documented. Post-infusion lymphocyte subset monitoring was variable but documented in 15. 1 patient was never B cell deplete. 9 never fully reconstituted. RTX dose information was available for 14. All received doses of 750mg/m2 with 6 having the dose capped at 1g. Dose frequency information was available for all. The majority (9) received 2 doses. Of the rest, 1 patient received a single dose. 3 received 3 doses, 2 had 4 and 1 had 5. All patients in whom information was available, received pre-medication with paracetamol, chlorphenamine and methylprednisolone (n = 14). 4 patients had side effects noted (rash/itch in 1, anaphylaxis in 1, facial flushing in 1 and post-infusion infection in 1). Clinical response was clearly documented in 8 patients, 7 of whom showed clinical improvement (2 had systemic jSLE, 2 had ANCA +ve vasculitis, 1 had isolated lupus nephritis, 1 had COPA syndrome and 1 had RF+ JIA). Conclusion Rituximab is used for a range of conditions and in a number of centres within SPARN. This review highlights variation in and poor documentation of pre-rituximab discussions and investigations, prescribing, monitoring and response. A SPARN rituximab protocol is being developed to address this. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Systemic autoinflammatory diseases (SAIDs) are rare, complex, and can often be debilitating. Establishing clinical and genetic diagnoses is equally complex and lengthy, causing significant stress to children and families. Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) recommendations emphasise the importance of access to genetic expertise alongside a tertiary paediatric rheumatology multidisciplinary team. Methods We established a 2-monthly combined clinic for children with diagnosed and suspected autoinflammatory conditions at Birmingham Children's Hospital (BCH). These are run by a consultant paediatric rheumatologist with special interest in unexplained periodic fever syndromes and autoinflammatory disorders, in collaboration with a consultant clinical geneticist interested in genetic causes for autoinflammation. The BCH paediatric rheumatology department operates a truly multidisciplinary approach with a collocated team. This allows ease of access to clinical nurse specialists, physiotherapy and occupational therapy, as well as effective provision of transitional care, a dedicated research nurse and research coordinator. To avoid multiple appointments and delays in diagnosis, upon receiving referrals for possible SAIDs, a standardised letter along with fever diary, blood forms and instructions for examining clinicians is sent to patients and the referring clinician. In establishing fevers and fever-patterns, possible evidence of inflammation and exclusion of infection prior to initial assessment, the process is streamlined and appointments reduced. A multi-speciality assessment with paediatric rheumatology and genetics is conducted, collaborating with immunology and other appropriate specialists. If SAID is suspected, appropriate panel testing is arranged, followed by whole exome sequencing if results are inconclusive. Patients are also recruited to national and international research studies if relevant, including BCH Rare diseases centre Stars Together Registry & NIHR BioResource rare diseases studies. Results In the past 3 years, the service has seen 96 new patients. Current patients have a variety of diagnoses, including: cryopyrin-associated periodic syndrome (CAPS)(NLRP3 mutations), familial Mediterranean fever (FMF) , TNF receptor-associated periodic syndrome (TRAPS), PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis), ADA2 mutation, NOD-2 mutation, Otulin-related autoinflammatory syndrome (ORAS), Aicardi-Goutieres syndrome (AGS), DNASE1L3 mutation, complement factor I deficiency, C2 deficiency, SLC29A3 mutation, Bechet's disease and undifferentiated SAIDs. This does not include patients who have primary immunodeficiencies, non-inflammatory vasculopathies or other SAID mimics. Drug therapies include: NSAIDs, short-term corticosteroids, Colchicine, Infliximab, Tocilizumab, Methotrexate, Adalimumab, Mycophenolate mofetil (MMF), Ruxolitinib and Anakinra, all supported by clinical nurse specialists. Conclusion This cohesive service provides single-point access to multidisciplinary paediatric rheumatology and genetics, along with national and international high quality research. A unified approach avoids the need for multiple appointments, allowing prompter diagnosis, initiation of appropriate treatment, and potentially earlier disease control. This aims to prevent disease-related damage, working towards optimal support for families and improving quality of life. A dedicated multi-speciality clinic also provides excellent training opportunities, both between specialities and for junior trainees. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background The need for training in adolescent and young adult (AYA) health is highlighted in paediatrics, adult medical trainees and the EULAR transition guidance. We aim to identify the current training needs of healthcare professionals regarding AYA health in a UK region including paediatric and adult rheumatology professionals. Methods An online survey designed using SelectSurvey.NET for healthcare professionals was distributed by email using a snowballing technique after piloting (1 nurse, 3 doctors). A control question was included on safeguarding, which is mandatory training for all healthcare professionals irrespective of setting. Results The overall response rate was 20% (166/820) and included 56% (n = 88/158 doctors -39% of whom were trainees), and 29% (n = 46/158) nurses. 8 identified themselves as rheumatology professionals (8/293, 3% rheumatology-specific response rate). 51% (n = 85/166) of healthcare professionals (including 2 rheumatology professionals) reported having received no formal training in AYA health (43% were doctors, 67% nurses). Of those who had previous training, the main training method was lecture based, with only 7% having accessed the UK Adolescent Health e-learning project. In comparison to 85% and 78% reporting adequate knowledge and skill respectively in safeguarding, less than 40% reported adequate knowledge and/or skills in key areas of AYA health (see Table 1). Respondents who had received training perceived themselves to have better knowledge and skills across many areas of AYA than those who had not received such training. Finally, in some areas of AYA health, there was a large discrepancy between perceived knowledge and skill level. P33 Table 1 Area of AYA Health  % of Healthcare Professionals with Adequate Knowledge  % of Healthcare Professionals with Adequate Skill      Rheumatology (n = 8)  Rest (n = 137)  Rheumatology (n = 8)  Rest (n = 126)  Safeguarding  87.5%  85%  75%  78%  Confidentiality & consent  75%  75%  75%  70%  Home/family  37.5%  61%  50%  48%  Health management  37.5%  56%  37.5%  52%  Transitional care  62.5%  54%  75%  47%  Eating  12.5%  54%  12.5%  40%  Exercise  25%  53%  25%  44%  Sleep  50%  43%  37.5%  40%  Peer relations  25%  43%  25%  34%  Safety   37.5%  40%  37.5%  29%  Mental health  12.5%  39%  25%  36%  Education/vocation  37.5%  34%  37.5%  27%  Sexual health  25%  35%  12.5%  28%  Social media  12.5%  33%  25%  30%  Substance use  0%  31%  0%  20%  Area of AYA Health  % of Healthcare Professionals with Adequate Knowledge  % of Healthcare Professionals with Adequate Skill      Rheumatology (n = 8)  Rest (n = 137)  Rheumatology (n = 8)  Rest (n = 126)  Safeguarding  87.5%  85%  75%  78%  Confidentiality & consent  75%  75%  75%  70%  Home/family  37.5%  61%  50%  48%  Health management  37.5%  56%  37.5%  52%  Transitional care  62.5%  54%  75%  47%  Eating  12.5%  54%  12.5%  40%  Exercise  25%  53%  25%  44%  Sleep  50%  43%  37.5%  40%  Peer relations  25%  43%  25%  34%  Safety   37.5%  40%  37.5%  29%  Mental health  12.5%  39%  25%  36%  Education/vocation  37.5%  34%  37.5%  27%  Sexual health  25%  35%  12.5%  28%  Social media  12.5%  33%  25%  30%  Substance use  0%  31%  0%  20%  P33 Table 1 Area of AYA Health  % of Healthcare Professionals with Adequate Knowledge  % of Healthcare Professionals with Adequate Skill      Rheumatology (n = 8)  Rest (n = 137)  Rheumatology (n = 8)  Rest (n = 126)  Safeguarding  87.5%  85%  75%  78%  Confidentiality & consent  75%  75%  75%  70%  Home/family  37.5%  61%  50%  48%  Health management  37.5%  56%  37.5%  52%  Transitional care  62.5%  54%  75%  47%  Eating  12.5%  54%  12.5%  40%  Exercise  25%  53%  25%  44%  Sleep  50%  43%  37.5%  40%  Peer relations  25%  43%  25%  34%  Safety   37.5%  40%  37.5%  29%  Mental health  12.5%  39%  25%  36%  Education/vocation  37.5%  34%  37.5%  27%  Sexual health  25%  35%  12.5%  28%  Social media  12.5%  33%  25%  30%  Substance use  0%  31%  0%  20%  Area of AYA Health  % of Healthcare Professionals with Adequate Knowledge  % of Healthcare Professionals with Adequate Skill      Rheumatology (n = 8)  Rest (n = 137)  Rheumatology (n = 8)  Rest (n = 126)  Safeguarding  87.5%  85%  75%  78%  Confidentiality & consent  75%  75%  75%  70%  Home/family  37.5%  61%  50%  48%  Health management  37.5%  56%  37.5%  52%  Transitional care  62.5%  54%  75%  47%  Eating  12.5%  54%  12.5%  40%  Exercise  25%  53%  25%  44%  Sleep  50%  43%  37.5%  40%  Peer relations  25%  43%  25%  34%  Safety   37.5%  40%  37.5%  29%  Mental health  12.5%  39%  25%  36%  Education/vocation  37.5%  34%  37.5%  27%  Sexual health  25%  35%  12.5%  28%  Social media  12.5%  33%  25%  30%  Substance use  0%  31%  0%  20%  Conclusion There is still a need to both raise awareness of and improve training opportunities for all healthcare professionals who interact with adolescents and young adults, including rheumatology. This training should be both practical and knowledge based to overcome the discrepancy between knowledge and skill levels. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Poster presentation Tuesday 8 October Background Juvenile idiopathic arthritis (JIA) is the most common childhood onset inflammatory arthritis. The last estimates of incidence of JIA in the UK are from nearly 30 years ago, prior to international classification consensus and the emergence of paediatric and adolescent rheumatology as a specialty. The aim of this study was to estimate incidence of JIA from primary care records in the UK since 2000. Methods The study used data from the Clinical Practice Research Datalink (CPRD), a database of UK primary care records broadly representative of the UK in terms of age, gender and ethnicity. A pre-defined list of JIA Read codes were used to identify incident cases annually from 2000-2018. Incidence rates (IR) with 95% confidence intervals(CI) were calculated and stratified by age and gender. The denominator was the population of CPRD <16 years old on the 31st December each year. Direct standardisation, applying Office for National Statistics population data, was used to estimate the UK IR. IRs in 5-year groupings were calculated 2000-2015 to identify change over time. Results Between 2000-2018, there were 1927 incident cases of JIA, from a total of 23,328,676 children <16 years old in CPRD. The total IR (95% CI) was 8.26 (7.90-8.64) per 100,000 population; for females and males respectively it was 9.83 (9.27-10.43) and 6.78 (6.33-7.27) per 100,000. Age-adjusted direct standardisation to the UK population estimated a total IR of 9.66 per 100,000 person-years. By age group, there appeared to be a lower incidence in middle childhood compared to early childhood and adolescents, as well as infancy where lower rates may be due to difficulties recognising the disease (Table 1). IRs over time did not appear to change. P01 Table 1: Incidence rates by age group Age (years)  Denominator  Cases  IR (95% CI)  Per 100,000  0-2  1739417  113  6.5 (5.40-7.81)  >2-6  5763003  534  9.27 (8.51 – 10.08)  >6-12  9464988  693  7.32 (6.80-7.90)  >12-15  6361268  587  9.22 (8.51-10.01)  Age (years)  Denominator  Cases  IR (95% CI)  Per 100,000  0-2  1739417  113  6.5 (5.40-7.81)  >2-6  5763003  534  9.27 (8.51 – 10.08)  >6-12  9464988  693  7.32 (6.80-7.90)  >12-15  6361268  587  9.22 (8.51-10.01)  P01 Table 1: Incidence rates by age group Age (years)  Denominator  Cases  IR (95% CI)  Per 100,000  0-2  1739417  113  6.5 (5.40-7.81)  >2-6  5763003  534  9.27 (8.51 – 10.08)  >6-12  9464988  693  7.32 (6.80-7.90)  >12-15  6361268  587  9.22 (8.51-10.01)  Age (years)  Denominator  Cases  IR (95% CI)  Per 100,000  0-2  1739417  113  6.5 (5.40-7.81)  >2-6  5763003  534  9.27 (8.51 – 10.08)  >6-12  9464988  693  7.32 (6.80-7.90)  >12-15  6361268  587  9.22 (8.51-10.01)  Conclusion This is the first study to provide contemporary UK estimates of the incidence of JIA for nearly 30 years. JIA was more common in females compared to males, and in early childhood and adolescence compared to other age groups. Incidence appeared to be stable over fifteen years. These data provide important information for patients, their families and healthcare providers; in addition, they are vital for appropriate resource planning and service provision in paediatric and adolescent rheumatology. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Poster presentation Tuesday 8 October Background We present four cases of patients on etanercept treatment for juvenile idiopathic arthritis (JIA) subsequently diagnosed with inflammatory bowel disease (IBD). We hypothesise that there may be identifiable characteristics of these patients which makes them more identifiable to the physician. Etanercept is a recombinant dimer of human tumour necrosis factor (TNF) receptor proteins bound to human IgG1. It is an effective second line treatment option in JIA for those who have failed, or are intolerant to, methotrexate, and an effective first line treatment option in those who have axial disease at diagnosis. Anti-TNF treatments are effective in IBD, however etanercept in particular does not have therapeutic benefit, and in one trial there was a non-significant trend towards worsening of disease. Methods Please refer to the Results section. Results Four cases, in a 15 year old male, 11 year old female, 10 year old female and 11 year old female, have been identified at our site. The time from etanercept initiation and diagnosis of IBD was between 2 weeks and 2 years 1 month. None of the cases had gastrointestinal symptoms at JIA diagnosis. All four were ANA negative, and in the two with information, both were HLA-B27 negative. Three out of four had hip arthritis involvement. There was a family history of IBD (ulcerative colitis) in only one of the cases. Aside from non-steroidal anti-inflammatory medications, there were no other immunomodulatory medications concurrently being taken in any of the cases while on etanercept. All four were anaemic (Hb < 120g/L) at diagnosis of IBD. Only one had raised ESR (43mm/hr) while all had normal CRP. Albumin was low in two (17g/L and 29g/L). Following gastroenterology review and endoscopy, two cases were diagnosed with Crohn's disease and two with ulcerative colitis. Conclusion It remains unclear whether etanercept plays a role in the development of IBD in these patients, whether it unmasks subclinical disease, or whether there is no association and it is simply a coincidence. However, there continue to be case reports and series suggesting an association between the two. We hope to identify further cases similar to these from sources such as the UK Biologics Registry and via surveys from teams at paediatric rheumatology centres throughout the UK. Further to the identification of cases, a retrospective review of patient characteristics will be carried out. This may allow for further determination of identifiable characteristics for these patients, and therefore suggest a subgroup of JIA patients with a higher risk of developing IBD, in which case caution when considering etanercept treatment would be advised. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Obesity levels have been increasing rapidly worldwide for the past two decades. The WHO has estimated the UK to be among the most overweight countries in the world, and according to government figures, the UK is now the fattest country in Europe with one in four adults obese. Obesity levels continue to rise within upcoming generations and if current trends continue, the forecast for the future health of today's children is bleak. Methods We aim to evaluate the body mass index (BMI) of children seen at the paediatric physiotherapy team. All new patients seen by a paediatric physiotherapist during the one month period June 2019 had their heights and weight measured at the first visit. The heights and weights of children were measured for all the children attending one physiotherapist's clinic. The BMI was calculated for these children using NHS BMI child calculator. Results A total of 28 new children attended the physiotherapist clinic. Of these, only 11 children were in the healthy BMI range and 17 were above the 90th centile. Among these 17 children, 12 were on the 98/99th centile. Ankle pain, lower back pain, knee or hip pain appeared to be the most common presenting symptoms of these children. Among the 28 children seen, 61% were above the 90th centile and 43% were on 98-99th centile. This data reflects only the children seen be one general paediatric physiotherapist. Children known to rheumatology team were seen by the therapists attached to the rheumatology team. Conclusion This audit highlights that children who are overweight and obese, experience more mechanical musculoskeletal symptoms. We conclude that further studies are needed to determine the epidemiology of musculoskeletal symptoms in children who are obese and overweight. The limitations of this project are small size and no collection of comorbidity data. As the trend of obesity is increasing, these issues should be addressed at every opportunity of contact with the health professionals. It is important, linking the weight issues to the conditions we are treating, and raising awareness to patient and carers. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background In November 2018 the paediatric rheumatology team in Belfast attended the Bridges self-management course. Bridges reflects on what works well as a team and how you can build upon current practice, with an emphasis upon a self-management approach. There are eleven Bridges principles aiming to help interdisciplinary team's co-ordinate their service around the patient. Bridges believes that the language we use can improve our clinical practice, especially with regards to problem solving, encouraging self-discovery, encouraging patients to reflect and enabling them to take action. Our team felt that we should use this approach to enhance our inpatient rehabilitation service. We chose this group of patients as we felt that this can be a challenging group to treat and it can take a long time to build supportive and trusting relationships. Methods Prior to commencing this project each member of the multidisciplinary team assessed the patient individually on the first day of admission. In retrospect we discovered that this led to repetition for the patient and this assessment was very clinician led. It also focused on clinician led (SMART) goals and achieving these. We furthermore realised we did not separately ask patients and parents what they wanted to gain from our service. Our method of improvement was to devise three questionnaires. One for a parent, one for a child under fourteen and over fourteen. These are distributed prior to being assessed by the team, allowing us to gather information and negating the need for repetition. The questionnaire gathers information regarding family members, education, school attendance, additional educational assistance, past medical history. For the patient we ask, what are their main concerns, what has helped so far, what support the patient has, what activities they enjoy as a family and what they hope to gain from their inpatient stay. The patient must fill out a description of their typical day, including their mood. Questions specifically for the parents are asking why their child has been referred, what their main concerns are, to outline their typical day, what do they feel has helped and what do they aim to gain from our team. Results We have utilised the questionnaires for every inpatient since January 2019. All team members feel that this new approach allows us to gain valuable information from patients and their parents thus encouraging them to adopt a self-management approach and to prioritise the patient's story. As a team we feel that we learn a lot about our patients using this method and that it is more time effective. It allows us to identify unrealistic hopes and discuss these. Conclusion We believe that using the Bridges approach and by mainly adapting our language skills and organisation we have improved this service. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background A previous audit carried out in 2017 showed that the blood monitoring of patients on methotrexate was substandard with over 60% of patients not being monitored according to guidelines. A positive correlation was found between postcode distance to the tertiary centre and substandard monitoring (those patients living further away showing worse monitoring records). As a result of this audit, a blood clerk was introduced in the team who would be responsible for chasing patients for blood results. This is a re-audit to ascertain the impact of the changes introduced to our service. Methods All rheumatology patients being prescribed methotrexate, etanercept and adalimumab were captured using dispensing records. The electronic prescribing software MEDITECHv6 was used to gather data for each patient including demographic data, frequency of blood tests and varicella and measles immune status. Patients were divided into two groups: methotrexate and biologics. These were further divided into treatment starters and maintenance patients. Treatment starters needed to meet the following 6 criteria in order to achieve appropriate monitoring: correct frequency of tests, full blood count (FBC) checked, liver function tests (LFTs) checked, renal function checked and varicella and measles immunity checked. Maintenance patients needed to meet 4 criteria to achieve appropriate monitoring: correct frequency of monitoring, FBC, LFTs and renal function. Potential correlations between substandard monitoring and postcode distance to the centre were explored. Blood clerk intervention rates and their effects were analysed. Results 216 patients were included in the audit. Overall 76 of them (35%) were appropriately monitored. For all patients on methotrexate and those on maintenance biologics, the main reason for substandard monitoring was a delayed timing of blood taking (50% and 65% of patients respectively). For patients starting on biologics the main factor affecting standard of monitoring was the lack of documentation of varicella and measles immune status (42% of patients). 72% of the patients with inappropriate frequency of blood tests received an intervention from the blood clerk. No correlation was found between distance to the centre and appropriateness of monitoring. Conclusion Adherence to monitoring guidelines has not significantly changed after the introduction of the blood clerk, with figures of adequate monitoring very similar to the previous audit (35%). The blood clerk contacts starting patients at 4 weekly intervals if needed, and maintenance patients every 3 months. Bearing in mind the time needed to set up a blood monitoring appointment after a blood clerk intervention, the number of patients with appropriate monitoring raises to 64% in the methotrexate group and 67% in the maintenance biologics group. This shows efficacy of the blood clerk intervention once it happens. Improved guideline adherence may be achieved by bringing forward the blood clerk interventions by 2 to 4 weeks in each patient group. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background An MDT-led educational class for parents of children newly diagnosed with JIA was developed with the aims of improving parental knowledge and confidence in managing a child with JIA. Our clinical observations were that a number of our patients had inaccurate ideas of what JIA was, three to six months into treatment. This is in-line with studies indicating that memory for medical information is often poor and inaccurate especially when the patient is anxious. Research also indicates the importance of patients developing an understanding of the condition and treatment which can aid in a number of improved outcomes including adherence. Our aim was to provide parents with an opportunity to increase their knowledge regarding JIA, addressing the medical and psychological management in a relaxed environment separate from a clinic appointment, without their children present. Several families had also requested to meet other parents for peer support and practical advice. Methods Parents of children newly diagnosed with JIA were invited to attend a session held in the evening at the NOC. Sessions were also conducted by the team at outreach clinics throughout the region. The programme was tailored to the child's developmental stage and included information about JIA and its management from the advanced nurse specialist, physiotherapist, occupational therapist, paediatric psychologist and an experienced parent. Prior to the session, parents completed a short questionnaire to assess their confidence in managing their child's condition and their understanding of JIA and treatment. Responses were measured on a ten point VAS scale. This was then repeated on completion of the session. Qualitative data regarding parent experience was also captured. Results Due to the large geographical area, attendance was approximately 50%. Parents' understanding about JIA and treatment on average increased 2.6 points on VAS scale, parent's confidence in managing their child increased on average 2.9 points. Parents reported the sessions were useful to explore what support is available, information regarding future management, importance of exercise, the emotional and psychological impact of JIA and how to manage it. 100% of parents reported their pre session aims were met. Conclusion Parental knowledge and confidence in managing their child's condition was shown to increase in all those who attended, demonstrated by increases in VAS scores. Sessions allowed parents to access information from specialist paediatric rheumatology professionals and meet with peers in a relaxed environment. Consideration is currently being given to the use of social media to boost attendance through a virtual session. We are planning to follow-up parents to see if the increase in VAS scores is maintained Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Delay in accessing specialist care is well recognised in childhood musculoskeletal conditions including juvenile idiopathic arthritis (JIA). Patients often describe circuitous routes within healthcare prior to diagnosis. In the UK, primary care is the gatekeeper to specialist services; review by a general practitioner (GP) is the first point of contact for the majority of non-emergency health concerns. The paediatric rheumatology service at the Great North Children's Hospital is the sole provider of paediatric rheumatology care in the North-East of England and North Cumbria seeing >600 new patients per year. Since 2012 the department has hosted an innovative GP integrated training post with a focus on new patient assessment and diagnosis. The success of this led to the appointment of a substantive GP post in 2017, providing independent new patient assessment for one clinic per week. The aim of this service evaluation was to review patient outcomes from this. Methods A mixed-methods approach was used. Quantitative data collected over a 12-month period included number of new patients assessed, time to first appointment, investigations, referrals, diagnosis and follow-up. Qualitative data was collected from patients (patient and parent satisfaction) and the multi-disciplinary paediatric rheumatology team (anonymised). Results 150 patients were assessed over the 12-month period, accounting for 25% of the new patient referrals to the service overall. Management included no investigations (29%), blood tests only (18%), blood tests and imaging (21%) and imaging only (32%). 87% of diagnoses made were non-inflammatory: Hypermobility (35%), mechanical pain (25%) and others (27%). 12% were inflammatory: JIA (4%), suspected connective tissue disease (3%), idiopathic uveitis (1%) and others (4%). 1 patient (1%) was unaccounted for as they failed to attend further appointments. Referrals were made for 59% of patients, the majority to physiotherapy. Mean waiting time to first appointment was 38 days (range 4 – 75). 67% of patients were discharged, with only 1 re-referral which resulted in the same diagnosis and repeated discharge. No complaints were received. Patient satisfaction (n = 19) was very good, excellent or outstanding in all domains and 100% were happy to be seen by a GP who works as a specialty doctor in paediatric rheumatology. Multi-disciplinary team (MDT) feedback was received from medical (n = 5), nursing (n = 2), physiotherapy (n = 1) and administrative staff (n = 2). The majority of domains were rated as excellent to outstanding with unanimously positive additional comments about the GP and trainees working within the service. Conclusion Integrating a GP into the paediatric rheumatology team has provided a significant contribution to new patient assessment and is highly rated by patients and the MDT. Unsurprisingly, the majority of new patient assessments are not diagnosed with inflammatory disease, and by providing our GP with appropriate training and support, such patients are able to be diagnosed and discharged appropriately. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Medication prescribing in paediatric rheumatology is closely monitored, particularly as a large number of medications used are either DMARDs or biologic therapy. Within our centre, we aim to give prescriptions when patients are attending outpatient clinics however due to long waiting lists, some patients require prescriptions before their clinic appointment. Currently, this is arranged by parents phoning in to our secretaries, however they often do not have the medication dose or route and this can delay efficient prescribing. We have also found that as most patients have their blood monitoring done in a primary care setting, we do not have access to their results. The aim of our audit was to formally assess outpatient prescribing in our centre, with a view to streamlining the process. Methods Data was collected over a one month period between 05/03/19 and the 05/04/19. Each prescription request was received by our CNS or secretary and passed to the NCHDs. The date and medication required was documented, along with chart availability, last clinic date, last bloods taken, whether the parents gave the dose and if a prescription was ultimately written. All data was analysed using Microsoft Excel. Results In total 36 prescriptions were requested. The majority of prescriptions (28) were routine prescriptions, with an additional 6 for 'emergency' prescriptions (e.g. diclofenac, ondansetron, NSAIDs and prednisolone) and 2 prescriptions were for a change in medication. Overall, 17 requests were for biologic agents, 7 for methotrexate and 5 for both biologic and methotrexate. Charts were available for 27 patients. Of those not available, 5 parents had given doses with their requests. If doses and charts were not available the dose from the last clinic letter on the system was used. Only in 10 cases out of 36, did parents leave a dose for medication with their prescription request. In total, 21 patients had recent blood tests recorded on our hospital database in the last 3 months and 27 had had clinic appointments in the preceding 6 months, with 13 of these occurring in the preceding 3 months. Conclusion This study highlights the large volume of external prescription requests received, as well as the potential for error in prescribing. We are currently planning on introducing a form that parents will be required to complete with all prescription requests, detailing dose and route of medication along with the results of their most recent blood tests. We hope that this will both streamline the process and allow for safer prescribing and plan to re-audit 3 months after its implementation. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Dacryoadenitis is an inflammatory enlargement of the lacrimal gland and can be idiopathic or associated with infections, malignant or inflammatory diseases like Sjögren's disease (SSj) or granulomatosis with polyangiitis (GPA). It remains the most common orbital inflammatory condition in children. This study purposes to describe a paediatric cohort of non-infectious dacryoadenitis. Methods Identification of children who underwent lacrimal gland biopsy at our centre between 2000 and 2018 was done by searching the database using two key-words: "lacrimal" and "dacryoadenitis". Patients with infectious or malignant dacryoadenitis were excluded. Medical notes were reviewed to collect data. Results Twenty-six patients underwent lacrimal gland biopsy at our centre between 2000 and 2018 and had inflammatory dacryoadenitis. 17 patients (65.4%) had non-specific chronic inflammation (NSCI) and 9 patients (34.6%) had an inflammatory systemic condition (Table 1). P53 Table 1 Patients' characteristics.   NSCI  GPA  Sarcoidosis  SSjogren  IgG4  Number of patients  17 (65.4%)  4 (15.4%)  3 (11.5%)  1 (3.9%)  1 (3.9%)  Female, n (%)  6 (35.3%)  4 (100%)  3 (100%)  1 (100%)  1 (100%)  Median age (Y) (IQ)  10.5 (4.9- 14.4)  9.72 (8.7- 13.0)  13.2 (11.45- 15.7)  11.9  11.2  Lacrimal gland involvement  Unilateral  13 (74.5%)  3 (75.0%)  2 (66.7%)  0  0  Bilateral  4 (23.5%)  1 (25.0%)  1 (33.3%)  1 (100%)  1 (100%)  Orbital symptoms            Ptosis  4 (23.5%)  1 (25.0%)  0  0  0  Erythema  3 (17.5%)  1 (25.0%)  1 (33.3%)  0  0  Pain  2 (11.8%)  1 (25.0%)  0  0  0  Extra-orbital involvement  Exocrine glands  0  0  1 (33.3%)  0  0  ENT  0  3 (75.0%)  0  0  0  Renal  1 (14.3%)  2 (50.0%)  0  1 (100%)  1 (100%)  Pulmonary  1 (14.3%)  1 (25.0%)  1 (33.3%)  0  1 (100%)  Myositis  1 (14.3%)  0  0  0  0  Arthralgia  1 (14.3%)  0  1 (33.3%)  0  0  Thyroiditis  1 (14.3%)  0  0  0  0  CNS  0  0  0  1 (100%)  0  Lymphadenopaties  0  0  0  0  1 (100%)    NSCI  GPA  Sarcoidosis  SSjogren  IgG4  Number of patients  17 (65.4%)  4 (15.4%)  3 (11.5%)  1 (3.9%)  1 (3.9%)  Female, n (%)  6 (35.3%)  4 (100%)  3 (100%)  1 (100%)  1 (100%)  Median age (Y) (IQ)  10.5 (4.9- 14.4)  9.72 (8.7- 13.0)  13.2 (11.45- 15.7)  11.9  11.2  Lacrimal gland involvement  Unilateral  13 (74.5%)  3 (75.0%)  2 (66.7%)  0  0  Bilateral  4 (23.5%)  1 (25.0%)  1 (33.3%)  1 (100%)  1 (100%)  Orbital symptoms            Ptosis  4 (23.5%)  1 (25.0%)  0  0  0  Erythema  3 (17.5%)  1 (25.0%)  1 (33.3%)  0  0  Pain  2 (11.8%)  1 (25.0%)  0  0  0  Extra-orbital involvement  Exocrine glands  0  0  1 (33.3%)  0  0  ENT  0  3 (75.0%)  0  0  0  Renal  1 (14.3%)  2 (50.0%)  0  1 (100%)  1 (100%)  Pulmonary  1 (14.3%)  1 (25.0%)  1 (33.3%)  0  1 (100%)  Myositis  1 (14.3%)  0  0  0  0  Arthralgia  1 (14.3%)  0  1 (33.3%)  0  0  Thyroiditis  1 (14.3%)  0  0  0  0  CNS  0  0  0  1 (100%)  0  Lymphadenopaties  0  0  0  0  1 (100%)  P53 Table 1 Patients' characteristics.   NSCI  GPA  Sarcoidosis  SSjogren  IgG4  Number of patients  17 (65.4%)  4 (15.4%)  3 (11.5%)  1 (3.9%)  1 (3.9%)  Female, n (%)  6 (35.3%)  4 (100%)  3 (100%)  1 (100%)  1 (100%)  Median age (Y) (IQ)  10.5 (4.9- 14.4)  9.72 (8.7- 13.0)  13.2 (11.45- 15.7)  11.9  11.2  Lacrimal gland involvement  Unilateral  13 (74.5%)  3 (75.0%)  2 (66.7%)  0  0  Bilateral  4 (23.5%)  1 (25.0%)  1 (33.3%)  1 (100%)  1 (100%)  Orbital symptoms            Ptosis  4 (23.5%)  1 (25.0%)  0  0  0  Erythema  3 (17.5%)  1 (25.0%)  1 (33.3%)  0  0  Pain  2 (11.8%)  1 (25.0%)  0  0  0  Extra-orbital involvement  Exocrine glands  0  0  1 (33.3%)  0  0  ENT  0  3 (75.0%)  0  0  0  Renal  1 (14.3%)  2 (50.0%)  0  1 (100%)  1 (100%)  Pulmonary  1 (14.3%)  1 (25.0%)  1 (33.3%)  0  1 (100%)  Myositis  1 (14.3%)  0  0  0  0  Arthralgia  1 (14.3%)  0  1 (33.3%)  0  0  Thyroiditis  1 (14.3%)  0  0  0  0  CNS  0  0  0  1 (100%)  0  Lymphadenopaties  0  0  0  0  1 (100%)    NSCI  GPA  Sarcoidosis  SSjogren  IgG4  Number of patients  17 (65.4%)  4 (15.4%)  3 (11.5%)  1 (3.9%)  1 (3.9%)  Female, n (%)  6 (35.3%)  4 (100%)  3 (100%)  1 (100%)  1 (100%)  Median age (Y) (IQ)  10.5 (4.9- 14.4)  9.72 (8.7- 13.0)  13.2 (11.45- 15.7)  11.9  11.2  Lacrimal gland involvement  Unilateral  13 (74.5%)  3 (75.0%)  2 (66.7%)  0  0  Bilateral  4 (23.5%)  1 (25.0%)  1 (33.3%)  1 (100%)  1 (100%)  Orbital symptoms            Ptosis  4 (23.5%)  1 (25.0%)  0  0  0  Erythema  3 (17.5%)  1 (25.0%)  1 (33.3%)  0  0  Pain  2 (11.8%)  1 (25.0%)  0  0  0  Extra-orbital involvement  Exocrine glands  0  0  1 (33.3%)  0  0  ENT  0  3 (75.0%)  0  0  0  Renal  1 (14.3%)  2 (50.0%)  0  1 (100%)  1 (100%)  Pulmonary  1 (14.3%)  1 (25.0%)  1 (33.3%)  0  1 (100%)  Myositis  1 (14.3%)  0  0  0  0  Arthralgia  1 (14.3%)  0  1 (33.3%)  0  0  Thyroiditis  1 (14.3%)  0  0  0  0  CNS  0  0  0  1 (100%)  0  Lymphadenopaties  0  0  0  0  1 (100%)  4/13 children tested had raised ACE (2 sarcoidosis and 2 NSCI). 7/17 had raised amylase (2 sarcoidosis,1 SSj, 4 NSCI). 3/16 had raised IgG (IgG4 disease, GPA and NSCI). ANA was positive in 4/19 patients (NSCI, GPA, Sarcoidose, SSj) and ANCA was positive in 4/19 patients (GPA). Thirteen patients received steroids (oral or IV) and elevan received DMARDS (MMF, methotrexate, azathioprine and hydroxychloroquine). Four patients received biologic (rituximab, adalimumab or infliximab). Six patients underwent orbitotomy. Patients still on follow-up had good response to treatment. One patient with NSCI presented with dacryoadenitis and later developed auto-immune thyroiditis and polymyositis, with positive ANA and anti-SRP antibodies. He was treated with steroids, methotrexate, MMF and IVIG. Conclusion Paediatric rheumatic inflammatory disorders have a diverse presentation and dacryoadenitis may be the initial manifestation. Biopsy is mandatory to exclude infection and malignancy. A full diagnostic workup is essential, especially if patients have positive ANA or ANCA. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Rituximab is a chimeric monoclonal antibody that depletes the B cell population by targeting cells bearing the CD20 surface marker and is used widely in the management of paediatric rheumatological conditions like juvenile systemic lupus erythematosus (JSLE), juvenile dermatomyositis (JDM), mixed connective tissue disease (MCTD) and juvenile idiopathic arthritis (JIA). Pneumocystis jirovecii pneumonia (PCP) is a potentially fatal opportunistic infection associated with congenital and acquired defects in T cell–mediated immunity. Our guideline did not recommend prophylaxis against PCP for patients on rituximab, unlike patients on cyclophosphamide, who are on cotrimoxazole until three months after cessation of the treatment. Cyclophosphamide is an alkylating agent which affects both B and T lymphocytes. Following the death of 16 year-old girl with JSLE due to PCP, the team reviewed the possible contributing factors, undertook a review of literature and discussed this at multi-disciplinary meetings involving the microbiology and immunology teams. This patient was found to have other risk factors for PCP – low CD4 T cells, concomitant use of corticosteroids and hypogammaglobulinaemia (IgG 3.0g/L). Although there is limited evidence that rituximab on its own increases the risk of PCP, there is emerging data that B cells may have a role in the protection against pneumocystis. Following the review, it was concluded that children on rituximab and an additional immunosuppressant (including corticosteroids) should receive prophylactic cotrimoxazole to cover PCP. Methods Retrospective audit carried out by the team to look at adherence to the new guideline regarding the use of cotrimoxazole for PCP prophylaxis in patients who have had rituximab between August 2017 and May 2019. Results P54 Table 1 Total number of patients who had rituximab  10  Number of patients who had other immunosuppressants concomitantly / recently (within previous 3 months)  7  Number of patients on rituximab monotherapy  2  Number of patients who are 6 months post-treatment  1  Number of patients with other risk factors for PCP  1 (hypogammaglobulinaemia)  Number of patients who are eligible for prophylaxis, as per the guideline  8 (7 for concomitant immunosuppression and 1 for hypogammaglobulinaemia)  Number of patients on cotrimoxazole  7 (87.5%) - one of the patients is on methotrexate, which is advised not to combine with cotrimoxazole  Total number of patients who had rituximab  10  Number of patients who had other immunosuppressants concomitantly / recently (within previous 3 months)  7  Number of patients on rituximab monotherapy  2  Number of patients who are 6 months post-treatment  1  Number of patients with other risk factors for PCP  1 (hypogammaglobulinaemia)  Number of patients who are eligible for prophylaxis, as per the guideline  8 (7 for concomitant immunosuppression and 1 for hypogammaglobulinaemia)  Number of patients on cotrimoxazole  7 (87.5%) - one of the patients is on methotrexate, which is advised not to combine with cotrimoxazole  P54 Table 1 Total number of patients who had rituximab  10  Number of patients who had other immunosuppressants concomitantly / recently (within previous 3 months)  7  Number of patients on rituximab monotherapy  2  Number of patients who are 6 months post-treatment  1  Number of patients with other risk factors for PCP  1 (hypogammaglobulinaemia)  Number of patients who are eligible for prophylaxis, as per the guideline  8 (7 for concomitant immunosuppression and 1 for hypogammaglobulinaemia)  Number of patients on cotrimoxazole  7 (87.5%) - one of the patients is on methotrexate, which is advised not to combine with cotrimoxazole  Total number of patients who had rituximab  10  Number of patients who had other immunosuppressants concomitantly / recently (within previous 3 months)  7  Number of patients on rituximab monotherapy  2  Number of patients who are 6 months post-treatment  1  Number of patients with other risk factors for PCP  1 (hypogammaglobulinaemia)  Number of patients who are eligible for prophylaxis, as per the guideline  8 (7 for concomitant immunosuppression and 1 for hypogammaglobulinaemia)  Number of patients on cotrimoxazole  7 (87.5%) - one of the patients is on methotrexate, which is advised not to combine with cotrimoxazole  We achieved 87.5% compliance in prescribing cotrimoxazole for PCP prophylaxis to all rheumatology patients receiving rituximab alongside another immunosuppressant agent; the one patient who this was not adhered to was due to potential adverse drug pharmacodynamic interaction between cotrimoxazole and methotrexate. Conclusion Although the current evidence points to increased risk of PCP in patients with inherited and iatrogenic defect of T cell function, there is emerging evidence that B cells may have a role too. Hence more work is required to determine the risk of PCP in patients on B cell targeted therapy (BCTT) and the need for prophylaxis. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Relapsing polychondritis in childhood is a rare multi-system disease characterised by inflammation of cartilage and can be life-threatening with complications of the larynx, trachea and cardiovascular system. It is often a delayed diagnosis with a more severe course than in adults; there is higher incidence of laryngotrachobronchial involvement, which can occur early and is often unresponsive to conventional therapy. Methods A previously healthy 14-year-old of African descent presented to a children's hospital with unilateral ear pain and swelling, lower anterior rib pain and red, painful eyes. Initial work-up showed microcytic anaemia and raised inflammatory markers. Initially treated with broad-spectrum antibiotics, markers of inflammation continued to rise and she became febrile. After discussion with tertiary paediatric rheumatology, polychondritis was suspected and high dose oral steroids were commenced, improving all symptoms. She was seen in tertiary paediatric rheumatology clinic and steroids were weaned. She quickly relapsed with fever, chest pain, cough, sore throat, dizziness and vomiting. On examination there was pain and swelling of the nasal bridge and cricoid tenderness. The left ear was swollen, warm and tender. She had tinnitus in the right ear. Further investigation showed high inflammatory markers, anterior uveitis and right sensorineural hearing loss. Management was pulsed intravenous methylprednisolone followed by oral Prednisolone as induction, with plans for methotrexate commencement. Parental concerns and missed appointments prevented methotrexate initiation; the family wished to withdraw steroids over two months before further treatment decision. On weaning steroids she developed stridor, barking cough, hoarse voice and dyspnoea. Saturations remained normal despite moderate work of breathing. Flexible nasendoscopy showed interarytenoid and subglottic oedema and likely trachobronchitis. She was commenced on CPAP, rituximab and a six-month course of cyclophosphamide. Due to parental concern and stress, relationships with healthcare professionals became increasingly challenging. Five months after initial presentation, she underwent semi-elective intubation owing to biphasic stridor and increased work of breathing. Subsequently extubation proved difficult and she was transferred to a specialist centre for ongoing management. Results The patient was subsequently extubated to BiPAP and completed cyclophosphamide infusions. Multi-speciality national teleconference agreed upon methotrexate and anti-TNF maintenance treatment with gradual wean of steroids. She has both saddle-nose and ear deformities, in addition to BiPAP-dependant airway stenosis. Discussions regarding airway reconstruction are ongoing with a national specialist team. Conclusion Relapsing polychondritis in childhood and adolescence is rare and more aggressive than in adults. This case demonstrates the speed destructive features can develop despite early treatment with steroids. Without aggressive and prompt treatment, life-threatening complications can develop rapidly and outcomes are poor. Also highlighted is potential airway reconstruction in collaboration with specialist centres. Excellent communication between healthcare professionals and with families is essential, especially when care of a complex and rare disease is shared between multiple centres. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Juvenile idiopathic arthritis (JIA) is the most common form of inflammatory arthritis in children and has an annual incidence of 33 per 100,000. With the introduction of biologic therapies, the goal of disease remission, both on and off treatment, is becoming increasingly achievable. Etanercept, a TNF-α inhibitor, was the first biologic to be used in JIA and is currently recommended as an add-on therapy in patients with inadequate response to methotrexate alone. While it is generally considered to have a good safety profile, a number of adverse events have been documented, including neuropathy. Methods We present two cases of etanercept related mononeuropathy, which persisted despite intravenous immunoglobulin (IVIG) therapy. Results Case 1: A 12 year-old girl with polyarticular JIA, who had commenced on etanercept 2 years previously, presented with an acute inability to fully flex her right index finger, associated with intermittent paraesthesia over the area. On examination, there was weakness in the deep flexor digit II and she was unable to flex at the distal interphalangeal joint. Deep tendon reflexes and sensation were normal. Nerve conduction studies showed absence of voluntarily recruited motor potentials in the right flexor digitorum profundus median to right digit II. Etanercept was discontinued and following review by a paediatric neurologist, a course of IVIG was commenced. To date, however, there has been no appreciable clinical improvement. Case 2: A 7 year-old girl with oligoarticular JIA, who had commenced on etanercept 3 years prior, presented with a ten week history of selective weakness in her left hand and absent power in her flexor pollicis longus. On examination, there was an apparent weakness in her flexor pollicis longus with 0/5 power. Sensory and deep tendon reflexes were normal. Etanercept was discontinued. EMG and nerve conduction studies definitively outruled a proximal median neuropathy or any median motor problem outside of the anterior interosseous nerve. EMG did not find incontrovertible evidence of motor conduction block however this remains a possible and indeed plausible mechanism. She was referred to paediatric neurology and a course of IVIG was commenced. Conclusion While etanercept remains a widely used medication in the setting of JIA, these case studies highlight the potential neurological adverse events that can occur. To our knowledge are the first reports of persistent isolated motor neuropathy secondary to etanercept use in the paediatric population. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Biologic therapies have revolutionised the management of patients with rheumatological conditions; with considerable cost to the NHS. The switch of originators to biosimilar products was challenging. We aim to share our experience of adalimumab biosimilar switch and believe this is reflective of the challenges experienced in some other centres. The development of biosimilar aims to challenge the excesses of pharma profits by encouraging competition in the market place. Previous switches to intravenous biosimilar products had been uneventful. We were informed that the trust was implementing switch of biologics to biosimilar and later told biosimilar A was the biosimilar version allocated to us. This was a decision beyond the Trust, with NHS England deciding on the allocation. For our Trust, biosimilar A was allocated for patients weighing 30kg requiring a 40mg dose and biosimilar B allocated for patients weighing less than 30kg requiring a 20mg dose (NB: no 20mg preparation available for biosimilar A). Patients commenced on biosimilar B 20mg preparation can remain on biosimilar B and move on to its 40mg preparation once they weigh 30kg. Biosimilar A, the cheaper of the two preparations, contains preservative, citrate. Both biosimilars are double the volume of the originator biologic. Case a biologic-naïve 16 year-old patient was the first to be treated with the chosen biosimilar A, and, upon self-administrating; the patient clearly expressed a high level of pain. Following the experience with the patient the team felt that this was not an appropriate option. Methods With the feedback from the patient, rheumatology team highlighted the issues and focussed on high quality patient centred care not just focussing on the short term cost saving option but understanding that the patient engagement and compliance is cost saving in the long run. The team enquired with other specialities and regional paediatric rheumatology centres regarding their biosimilar switch. Results Liaising with the High cost pharmacy team, we successfully obtained authorisation to prescribe biosimilar B. We promptly switched the patient to biosimilar B and prescribe this now for all suitable to switch and new patients. So far there have been no negative comments or issues from the switch to the citrate free product. Conclusion It is vital that we provide an evidence based cost saving medication to treat children with long term conditions. However when new medications are being introduced, there needs to be greater consideration for the paediatric population which currently seems lacking. Collaborative working with the high cost pharmacy team, regular communications with other specialities, adult colleagues and involving the national nurse specialists group greatly benefitted us in managing this situation. More studies are required to identify patient factors involved in these scenarios. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background We present a case that was referred and seen in our chronic pain clinic, and highlight the importance of keeping an open mind about alternative diagnoses when reviewing these patients. Methods Please refer to the results section. Results A 12 year-old girl was referred with right sided neck and shoulder pain associated with occasional pins and needles. The patient was a competitive swimmer, training and competing five times a week at her peak. She did not have a history of mottling or colour change, and clinically she had full range of motion of her joints and no neurological deficit. On repeated assessments, she had reproducible pain on horizontal flexion and reduced muscle bulk of the right shoulder, although power was preserved. Functionally the patient had to reduce her participation in swimming due to pain, and found it difficult to carry or lift things, brush or wash her hair, or write for extended periods of time. Imaging with shoulder X-ray and shoulder and cervical spine MRI did not reveal a cause, and she did not have an anatomical variation such as a thoracic rib. Due to the reproducibility on swimming, an ultrasound looking at the insertion of the biceps insertion during dynamic movements was arranged and was also normal with no subluxation noted. As she failed to improve with chronic pain education, chronic pain approach to physiotherapy sessions and graded reintroduction of swimming, ultrasound angiology was arranged. This showed minor disease in the right subclavian, axillary, brachial, radial and ulnar arteries, with significant reduction in right brachial arterial flow with the patient supine, right arm in horizontal flexion and contralateral head turn. This suggested arterial thoracic outlet syndrome (TOS). All other positions resulted in no significant difference in arterial flow. TOS is traditionally broken into two categories – neurogenic and vascular, the latter can be broken down further into arterial or venous. While in adults, the vast majority (90-95%) present as neurogenic, in the paediatric population there is a higher proportion of vascular cases. This has implications, as vascular TOS can be complicated by vessel disease and thrombus formation. There are a number of case reports in which management is reported as decompression of the thoracic outlet with partial first rib resection, with or without scalene muscle resection. Consideration of hypercoagulable states is also warranted. We have referred our patient to the vascular team for an urgent review and await their assessment and feedback about management. Conclusion A proportion of patients managed with chronic pain syndrome will have an alternative explanation for their pain. In particular, if a patient has focal pain or pain that is reproducible on a particular movement or activity, consider alternate diagnoses. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Very little literature exists in supporting the assessment and management of cystic fibrosis related arthritis (CFA) particularly in paediatric and adolescent cases. Methods A 12 year-old male with cystic fibrosis (CF) was admitted via A&E with severe joint pain that was thought to be associated with a respiratory infection and was treated accordingly. Repeated exacerbations of joint disease occurred every 1-2 months, lasting approximately 1 week with significant joint pain, stiffness, and reduced function. The patient did not feel his exacerbations of CFA were related to his respiratory symptoms, admitting he under reported arthritis symptoms to avoid being admitted for IV antibiotics. Initial rheumatology review resulted in treatment with IV antibiotics and piroxicam. Flares continued, prompting his CF physiotherapist and multidisciplinary team to seek further specialist review for his now longstanding symptoms. He scored 16 out of 50 on the McGill Pain Questionnaire (MPQ) with a visual analogue score (VAS) of 46 out of 100. At 16 years old he was seen by an MSK physiotherapy and rheumatology team working within a wider CF team. He had a 4 year history of symmetrical joint pain affecting knees, wrists and hands, on a background of bronchiectasis with previous non-tuberculous mycobacteria infection, cystic fibrosis related diabetes, and poor nutrition. He reported some episodes being so bad he was unable to get out of bed, and struggling with the stairs at college. He had 6 swollen and 8 tender joints including wrists, MCPs and knees. Ultrasound imaging of the wrists and hands showed confirmed active synovitis with grade 3 greyscale changes and grade 2 power Doppler. Hydroxychloroquine was commenced with naproxen as required, and wrist splints were provided. Results At follow-up he reported significant symptomatic improvement with occasional minor stiffness in wrists and knees which responded quickly to naproxen and was no longer limiting his activities. Repeat ultrasound confirmed improvement with no active power Doppler, but some ongoing grade 1 greyscale changes and a small wrist effusion. He has returned to previous function with an MPQ score of 0, and VAS of 10 out of 100. He reported improved quality of life due to reduced pain and fewer, shorter, and more manageable exacerbations. Conclusion Specialist review and commencement on disease modifying anti-rheumatic medication helped to manage CFA in this case. All cases of suspected CFA should be referred for specialist rheumatology opinion. CF physiotherapists are in an excellent position to identify cases and empowering the multidisciplinary team has been vital in this case. Further trials are required to investigate optimal management of CFA, but it seems likely that directly addressing the arthritis with disease modifying therapy may play an important role. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Sarcoidosis is a rare autoinflammatory systemic granulomatous disease that can affect any organ in the body. Paediatric literature does not give a prevalence of neurosarcoidosis. Clinical features can be tricky to identify and piece together to suggest the diagnosis and biochemical markers such as serum angiotensin converting enzyme lack sensitivity. Methods This case report of a 13 year-old boy demonstrates his journey to diagnosis as he bounced between multiple private and NHS care teams. Referral was to our paediatric rheumatology department with a single oesophageal granuloma and bilateral uveitis. Symptoms include nausea, vomiting, weight loss which was regained, fatigue, disturbed sleep, chronic dry cough, then worsening headaches. He had been treated privately with steroid eye drops for bilateral uveitis. Histology of the oesophageal granuloma showed no acute inflammation. A private paediatric rheumatologist review concluded idiopathic uveitis with no underlying systemic disease that could be delineated at that time. A private haematologist subsequently diagnosed him with a chronic systemic inflammatory process leading to an iron deficient anaemia. Results He represented to his ophthalmologist with a 3 week history of worsening headaches and was found to have papilloedema. He was urgently jointly reviewed in the NHS setting by a paediatric gastroenterologist, neurologist and rheumatologist. Clinically apart from the bilateral pappiloedema there were no signs of disease. Urgent MRI demonstrated widespread inflammation and oedema in both cerebellar hemispheres and within his white matter bilaterally, as well as some cortical patches. Magnetic resonance arteriogram and magnetic resonance venogram were normal. Tonsillar herniation and ventricular tightness due to raised intracranial pressure was noted. He was admitted urgently. Bloods showed normal inflammatory markers, iron deficient anaemia, ANCA negative, ACE 30 iu/L, Quantiferon negative. CXR was normal as were pulmonary function tests. A raised urinary calcium with raised calcium:creatinine ratio led to a renal tract ultrasound study that showed early nephrocalcinosis bilaterally. Ophthalmology review demonstrated granulomatous pan uveitis with macular oedema. NOD2 genetic testing was negative. Conclusion Neurosarcoidosis was diagnosed. He was treated with 2mg/kg IV Methylprednisolone over 3 consecutive days then a slow prednisolone wean starting at 60mg per day orally and 6 times a day Prednisolone eye drops. Weekly methotrexate 15mg subcutaneously and 5mg folic acid were started followed shortly by subcutaneous Adalimumab 40mg fortnightly. Repeated MRI brain scans after treatment commenced showed resolution of cerebral inflammation. Follow up ophthalmology reviews demonstrate resolution of his uveitis. Bilateral nephrocalcinosis has persisted despite normalised serum and urinary calcium. It was not until he was seen by an ophthalmologist with expertise in both areas that the severity and nature of the uveitis including it being granulomatous was picked up. The diagnosis was delayed due to the lack of joined up working between private and NHS sectors. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Liver injury is known in patients on methotrexate (MTX). We retrospectively reviewed records of patients who had liver biopsy for suspected MTX induced liver injury and identify risk factors if any. Methods Children (≤ 16 years) referred to us from 2002 to 2015 with suspected MTX induced liver injury were identified and data related to their underlying diagnosis, biochemical parameters, Body Mass Index (BMI), dose and duration of MTX at the time of liver biopsy, use of concomitant drugs, duration and other causes for elevated transaminases, liver histology including clinical decisions to continue or stop MTX were extracted from the data base. All the liver biopsies were re-analysed for this study and scored using the Roenigk classification by a single histopathologist blinded to patient characteristics. Results Seventeen liver biopsies were performed in 14 children (3 had repeat biopsies) who were referred for frequent/persistent transaminitis (> 2 times upper limit normal). The base line bilirubin, transaminases and synthetic liver function were normal in all prior to MTX therapy. All were on MTX at the time of biopsy except two who had been off the drug for 3 and 8 months following frequent transaminitis. They had liver biopsy to guide reintroduction of MTX in their treatment regime. None of the patients had renal impairment, diabetes or underlying liver disease. Thirteen biopsies (82 %) had none (n = 6) to mild liver injury (n = 7) and were advised to continue MTX. Four biopsies (18%) showed significant liver injury (grade 3b and 4). Of these 3 patients were advised to stop MTX while one was advised to continue and repeat liver biopsy in 6 months. He however, stopped MTX soon thereafter. All biopsies of children (n = 3) with BMI ≥ 91st centile had liver damage. Two of thirteen biopsies with BMI < 91 centile had moderate-severe damage. No correlation was seen between the histologic findings or their severity with dose or duration of MTX, concomitant use of other drugs, severity/highest transaminase peak, total duration of transaminase abnormality, longest single episode of transaminase abnormality or frequency/number of peaks of abnormal transaminases. Of the 3 with repeat biopsies, fibrosis had progressed in 2, necessitating discontinuation of MTX in 1. Conclusion The finding of significant liver injury in our cohort is not entirely in keeping with previous paediatric studies which report non-significant fibrosis or cirrhosis. We could not identify any risk factors though children with BMI ≥ 91st centile seemed more likely to have liver injury. This finding is similar to adult studies implicating obesity as risk factor though it is difficult to draw definitive conclusions given the small size of our cohort. Other limitations include the retrospective nature of the study, bias as MTX dose modifications and referral were at the discretion of the rheumatologist and no information on alcohol intake in the teenagers. This study emphasises the need for careful monitoring to prevent severe fibrosis and cirrhosis. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background IgG4-related disease (IgG4RD) is an immune-mediated fibroinflammatory condition characterized by the infiltration of IgG4-carrying plasma cells and storiform fibrosis in most of the tissues. The condition is reported to cause multisystem involvement, however salivary gland is the most commonly affected organ with IgG4-related sialadenitis. Raised IgG4 concentrations in the serum and prominent infiltration by plasmacytes expressing IgG4 in the lacrimal and salivary glands have been confirmed. Methods We conducted a retrospective case review of a 10 year-old girl who was diagnosed with IgG4-RD at Great Ormond Street Hospital for Children in 2017. Patient's clinical, laboratory and imaging data was extracted from our database and literature search was done to find out different manifestations of the IgG4-RD in Children. Results 10 year-old-girl with lacrimal and salivary gland swelling, sicca symptoms and fatigue. Ultrasound scan neck revealed multiple small lymph nodes and enlargement of both submandibular glands (Picture1) Salivary glands also appeared bulky and heterogenous with multiple small hypoechoic focci. Appearances likely to represent sialoadenitis and there was no evidence of malignancy or lymphoma. USS abdomen normal. Full blood count, routine biochemistry and urine microscopy normal. Autoantibodies came back negative (ANA: Negative, ANCA: Negative, Anti-Ro and Anti-La: Negative, RF:Negative, Thyroid autoantibodies:Negative) IgG level elevated (in repeated samples). IgA, IgM and IgE levels normal. IgG subgroups revealed significantly elevated IgG4 levels (21.49 Normal range: 0-1.1) IgG1, IgG2 and Ig G3 levels elevated as well. Lymphocyte subsets were normal. The biopsy of salivary gland: Chronic inflammation with IgG4 staining, suggestive of IgG4 related disorder. Lung function test showed decreased DLCO at 76 %, FEV1 at 82% and FVC at 89% suggestive of interstitial lung involvement. CT Thorax: Multiple abnormalities including moderate lymphadenopathy, renal parenchymal lesions and interstitial lung abnormality. MRI Abdomen: T2 hypointense renal foci, suggestive of infiltration as part of the IgG4 related disease. Diagnosed with IgG4 related disease. Treatment started with intravenous methylprednisolone followed by Anti-CD20 (Rituximab) therapy and a weaning plan for steroids. Mycophenolate mofetil commenced for the maintenance therapy. Patient has shown good response with clinical and lung function improvement. Conclusion IgG4-RD is a rare condition which can cause multisystem involvement with the infiltration of IgG4-bearing plasma cells in the tissues. We wanted to emphasize that this condition could also be seen in the paediatric population. We also wanted to highlight that after further investigations multisystem involvement can be discovered. Steroids are mainstay of the treatment with Anti-CD20 medication (Rituximab) and steroid sparing agents such as mycophenolate mofetil could be the choice for maintenance therapy. However, further studies need to be done in paediatric age group. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background 1:1 art therapy sessions have been offered to rheumatology patients at the Royal Hospital for Children, Glasgow since 2012, supported by the Teapot Trust. A relationship is established between child and Therapist, allowing safe exploration of difficult issues. We describe a new way of collaborative working to include input from other professionals within therapy sessions. Methods A 10 year-old girl was referred to art therapy by the consultant rheumatologist to help her process an existing diagnosis of Marfan syndrome. She displayed intense anger towards her family, lost all confidence socialising and became extremely distressed when having medical examinations. The child identified anger towards her family was because she perceived a lack of honesty about her condition making her feel worried she would die, which in turn prevented her from socialising. The child noticed she became distressed when clinicians didn't ask permission to examine her. It was apparent that the child needed a joined up approach with the art therapist and consultant rheumatologist collaborating to communicate the child's views to all professionals involved, which at times involved the consultant rheumatologist joining art therapy sessions. The child identified her own goals for art therapy: Not saying mean things when I'm angry and think of other ways to express my anger Get my confidence back Progress in therapy was measured using Goal Based Outcomes and Psychlops Outcome Measurement Tools allowing the child to report on her own progress. Results Through Goal Based Outcomes, she communicated her anger 'was getting better' and she felt confident enough 'to go on a trampoline'. Family sessions were used to help the child speak honestly with her parents about her diagnosis and prognosis. Having her consultant rheumatologist at sessions enabled her to ask questions like 'why do I have this,' 'will I die' and 'can I go on roller coasters.' Through Psychlops, she communicated her anger was related to feeling embarrassed and exposed during examinations. The art therapist supported the child to explore her body boundaries and to create rules clinicians should follow when examining her. The consultant rheumatologist shared these rules with the rheumatology MDT. The MDT encouraged the child to create a poster to be displayed in the paediatric outpatient clinic, which raised her confidence. At subsequent medical examinations she has been able to communicate her wishes to clinicians instead of becoming distressed. The child is also socialising more and can communicate her feelings at home without becoming angry. Conclusion This collaborative approach supported the child to process her diagnosis by creating a safe and honest space for her feelings to be explored whilst reducing barriers to engaging with clinicians. We have since used this methodology with other members of the rheumatology MDT. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Composite disease scores in juvenile idiopathic arthritis (JIA), such as the clinical Juvenile Arthritis Disease Activity Score (cJADAS), include multiple disease manifestations, presented as a single score. These overall scores aid understanding of disease holistically in each child or young person (CYP), and have been suggested as outcomes for clinical trials and targets in treat to target clinical strategies. However, signs and symptoms of disease may not follow similar patterns following a JIA diagnosis. It is not currently known what the patterns of disease activity are in CYP with JIA and how these cluster over time. Methods CYP with JIA were selected if enrolled in the Childhood Arthritis Prospective Study (CAPS), a UK multicentre inception cohort, before January 2015. cJADAS10 components (active joint count 0-10, physician global, patient/parent global) were collected at diagnosis, six months, one year and then annually to three years. Multivariate group-based trajectory models modelled cJADAS10 component scores using censored-normal (physician and parent global) and zero-inflated Poisson (active joint count) distributions. Within linear, quadratic and cubic polynomials, one to ten trajectories were tested. The optimal models were selected using Bayesian Information Criteria, model parsimony and clinical plausibility. Results Of 1,183 CYP selected, the majority were female (65%) and of white ethnicity (90%) with oligoarticular JIA the most common JIA category (45%). The optimal model identified six multivariate patterns of disease. In four of these clusters, signs and symptoms of disease had similar patterns over time: Low-Remission (32%), Low-Low (20%), High-Low (16%) and High-Low-High (10%). However, in two groups, Low-Chronic (14%) and High Chronic (8%), manifestations of inflammation and wellbeing followed different trajectory severities and shapes over time. These groups demonstrated persistent poor wellbeing despite control of inflammatory signs. Conclusion Disease activity in CYP with JIA does not improve in a uniform manner following initial presentation to paediatric rheumatology. Six latent multivariate trajectories have been identified in young people with JIA, two of which persist with chronic poor wellbeing despite lowered inflammation. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background An estimated 10-20% of all patients with systemic lupus erythematosus (SLE) develop clinical disease before the age of 18 years and are therefore classified as juvenile-onset SLE (JSLE). JSLE patients have a higher prevalence of lupus nephritis (LN) compared to adult-onset SLE and decreased long-term survival compared to JSLE patients without LN. We aimed to identify clinical and laboratory predictors of LN in JSLE patients by comparing the baseline characteristics of JSLE patients with and without LN. Methods This is a single-centre retrospective study that included JSLE patients reviewed in our young adult and adolescent clinics after transitioning from paediatric services. All data was analysed descriptively. Mann-Whitney U or Chi-square test were performed to compare the characteristics between patients with and without LN. In addition, we performed uni- and multivariate analysis to investigate potential predictor biomarkers for LN. Results We identified 134 JSLE patients, 45 (34%) of which had LN. 112 (83.6%) of the patients were female and the median age was 22 years (interquartile range [IQR] 20- 24). The median disease duration was longer in the patients with LN (10 years [IQR 8- 14]) in comparison with the patients without LN (8 years [IQR 6- 13]), p = 0.012. As expected, the highest dsDNAlevels ever were observed in patients with LN (p = 0.022). The overall clinical manifestations and complications did not differ between JSLE patients with or without LN, except for the prevalence of low bone mineral density. 16 (35.5%) patients with LN had low bone density and only 11 (12.4%) patients without LN (p = 0.008). Factors associated with low bone mineral density in a univariate logistic regression analysis were: the use of mycophenolate mofetil (p = 0.021) and the presence of LN (p = 0.007), while LN also associated with low bone density (p = 0.008) in a multivariate analysis. Skin involvement was the most common clinical manifestation in both JSLE patient groups. Treatment was more aggressive in patients with LN, including the use of cyclophosphamide, mycophenolate and rituximab, but there was no difference regarding the length of the steroid treatment. The majority of patients (67%) had one flare of nephritis. For the remaining patients, the number of flares ranged from 2 (13%) to 12 (2.2%). The class of nephritis was reported in 34 out of 45 patients, and focal lupus nephritis (class III) was the most common type (44%) reported. There were no statistically significant differences in the baseline characteristics or treatments among the different classes of LN. Conclusion Low bone mineral density in patients with JSLE is well described. However, this is the first study highlighting the association of low bone density with LN independent of total duration of steroid treatment or other clinical manifestations. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Chronic idiopathic musculoskeletal pain (CIMSKP) has become increasingly recognised as a major cause of morbidity in adolescents. In view of increasing referrals to a paediatric rheumatology centre, the Learning and Exercise to Avert Pain (LEAP) intervention was designed as an approach to pain management within a group setting, led by an occupational therapist and a physiotherapist. It involves 2 hour weekly sessions for 6 weeks and includes education, gym activities and relaxation sessions with routine collection of Pain VAS, CHAQ, Bath Pain questionnaires (adolescent BAPQ and parent BAPQ-p forms) at baseline, end of LEAP and 3 months follow-up. We aim to assess the feasibility and impact of LEAP for adolescents with CIMSKP. Methods Young people who had been referred for the LEAP programme were identified (n = 46). Basic demographics and questionnaire scores were collected from electronic patient records and therapy notes. Feasibility was assessed by delivery of programme and percentage completion of questionnaires at three time-points. Semi-structured interviews were conducted with healthcare professionals involved in the programme and analysed using thematic analyses as were any free text comments on the BAPQ from adolescents and parents. Statistical analysis of the outcome data was performed using paired t-tests and SPSS software (v22). Results 46 adolescents were identified: 85% female, median age 16 years (range 10 to 19). 34 participated in LEAP, 12 received 1:1 therapy. Feasibility of programme delivery was achieved with completion of all six sessions. Completion rates for all 3 measures for adolescents/parents at baseline, end of programme review and follow up were 78.6/71.4%, 64.3/71.4%, and 45.5/36.4% respectively. Adolescent scores indicated a significant increase in impairment in the development subscale scores on BAPQ from baseline to programme end (p = 0.021) although not at follow up. Parent scores for adolescent anxiety and pain-specific anxiety on BAPQ-p increased significantly from end of programme to follow-up (p = 0.017, p = 0.034 respectively). Parents scored higher than adolescents for most outcomes. The qualitative data (available for 11 adolescents, 12 parents) revealed that parents and adolescents appreciated learning coping techniques and gaining a greater understanding of chronic pain. The intervention was perceived to be socially beneficial in particular but imperfect in terms of data collection by the professionals interviewed (n = 2). Conclusion Baseline demographics of the LEAP participants echoed current literature. Delivery of the programme was feasible and the programme was positively received by young people, their parents and professionals alike. The increase in adolescent development subscale scores following the programme could represent increased self-reflection and/or peer to peer observations as a result of the group setting but needs longer term follow-up data to clarify further. The increase in parental anxiety highlights the need for a concurrent session for parents. Routine collection of objective measures remains challenging in non-research setting. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Juvenile idiopathic arthritis (JIA) is the commonest cause of uveitis in children. Uveitis is a significant cause of visual impairment in children with potential complications such as band keratopathy, cataract, posterior synechiae, glaucoma, cystoid macular oedema and a retinal problems. Following the initial treatment with topical and systemic corticosteroids, a wide variety of immunosuppressant agents have been used in the treatment of non-infective uveitis including methotrexate (MTX) and biologic therapy, most of which are administered parenterally. Mycophenolate Mofetil (MMF) is an oral immunosuppressant that inhibits the proliferation of T and B lymphocytes, which has been tried in children with uveitis with mixed results. We started using MMF in children seen in our paediatric uveitis clinic initially as a third line agent when the inflammation is not well controlled despite therapeutic doses of MTX and Adalimumab, as the NHS England Clinical Commissioning Policy does not recommend the use of Infliximab in uveitis not associated with JIA. Buoyed by some favourable result, we have since then used MMF as second line and even first line agent in children with uveitis. Methods Retrospective analysis of the clinical profile of children with uveitis on MMF at a tertiary paediatric rheumatology centre with focus on response to treatment. Results Of the 8 patients who received MMF, six (75%) were girls. Half of them (4/8) had idiopathic uveitis and the rest, associated with JIA. 2/8 patients had MMF as third line agent on top of MTX and Adalimumab, while five of them had it as second line agent on top of Adalimumab due to either MTX intolerance or needle phobia. One patient was started on MMF as first line agent following topical steroids. 6 (75%) of the patients responded/stayed in remission following the addition of/switch to MMF. Conclusion MMF has shown initial promise in the treatment of uveitis in children with uveitis in this small cohort, in line with some existing evidence. It was initially used in patients who were not keen on injections/intolerant to MTX or had failed all existing options. This is a small cohort of patients and we would welcome more research in this area. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background The BSPAR section council consensus-based guidance is the most widely used guideline on the use of methotrexate. This recommends that varicella immunity status is checked prior to commencing methotrexate and to consider immunisation if the child is non-immune. It also suggests considering checking measles status and testing for TB in high risk patients. We aim to evaluate our practice of screening paediatric rheumatological patients prior to commencing them on methotrexate. All patients in our centre have methotrexate teaching with a clinical nurse specialist prior to commencing treatment. Methods This study included paediatric patients who were prescribed methotrexate in all forms (subcutaneous, intravenous and orally) for rheumatological conditions and uveitis during a 2-year period (2016 – 2018) regardless of when it was first commenced. Digital health records were accessed to obtain the data retrospectively. Patients were excluded if they had been started on methotrexate prior to joining our centre, or were lost to follow up during the study period or if the required data was unavailable. Results 123 patients were identified to have been prescribed methotrexate during the 2-year study period and 102 patients were included in the study. 28 patients were commenced on methotrexate during 2016 /17 and others were commenced anytime between 2008 -2015. 91 (89%) patients had their varicella status checked prior to commencing methotrexate and 7 patients were checked after treatment was commenced. It was not checked in 4 patients (1 sample rejected and not repeated). Of those checked 20 patients were non-immune and 16 were vaccinated. 57 patients had had measles serology checked, 31 of these prior to commencing methotrexate. 35 patients had TB Quantiferon checked prior to methotrexate being started and 39 after. There were no positive results and 6 (8%) indeterminate of which 5 were repeated and 4 were negative. Of the indeterminate results 4 samples were taken prior to commencing methotrexate and 2 after. Conclusion This study shows that local adherence to checking varicella status prior to commencing methotrexate and vaccinating non-immune patients is good. Routine practice for checking measles, hepatitis and TB is varied. The data was captured over a 2-year period but actually included practice of methotrexate commencement over 9 years and thus time span and change in doctors will have contributed to the variation in practice. We believe some results from outreach clinics were not captured. All four tests are often carried out if it is felt the patient is likely to go on to require biologics. An updated guideline would help streamline the pre-DMARD screening and may limit the number of unnecessary investigations. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Peer support, which can take many forms, involves people sharing knowledge, experience and practical help. It is recognised as a useful mechanism for families living with children who have a long-term condition, such as juvenile idiopathic arthritis (JIA). It is often provided by voluntary organisations. While some forms of peer support were established for families living with JIA in the United Kingdom (UK), there were no local support groups in a number of locations. Given that over 12,000 families are living with JIA, the need for access to peer support for families became evident. Therefore, the aim was to establish one local support group for JIA families in London; while identifying whether any other families were organising similar activities elsewhere in England and Wales. Methods The initial concept was discussed and endorsed by a group of paediatric rheumatology clinicians and the British Society for Paediatric and Adolescent Rheumatology (BSPAR – part of the British Society for Rheumatology) Parent Group. Policies and guidelines for the network and local support groups were then created. The Juvenile Arthritis Parents and Families UK Facebook group were consulted via a parent-led request to identify who lived in the London area. Subsequently, a JIA Matters North and West London closed Facebook group was created, and families from the area were invited to join. Once several members had joined, the JIA Matters Co-ordinator arranged a local, face-to-face meet-up for families. Using this model, paediatric rheumatology centres across different counties in England and Wales were approached by JIA Matters to recommend parents/carers who could be suitable parent/carer representatives to lead on establishing local groups. Results Between July 2017 and March 2019, 36 JIA Matters groups were established across England and Wales. Some groups have rapidly developed, while others remain conservative. Attendance at local, face-to-face meet-ups have been variable, with some groups experiencing difficulties in encouraging people to attend. The first local, face-to-face meet up of the NW London group was attended by seven families. Some paediatric rheumatology centres have been responsive and proactive in using JIA Matters and have begun to engage with the JIA Matters Coordinator to help enhance family engagement within local activities. To aid the sustainability of the network across the country, JIA Matters was incorporated into the Children's Chronic Arthritis Association (CCAA) in March 2019, strengthening the national reach of the network to help ensure families across the country have access to local peer support. Conclusion JIA Matters has demonstrated how parent-led initiatives on a local level can indeed influence activities on a national level. From modest aims, JIA Matters has evolved to connect families living in the same geographic area, while promoting family engagement in research, service development and activities within paediatric rheumatology centres. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Tocilizumab (TCZ) is a monoclonal antibody that targets the interleukin 6 receptor. It is approved for use in children over the age of 2 years with systemic onset juvenile idiopathic arthritis (SoJIA) and polyarticular juvenile idiopathic arthritis (pJIA) who have not responded to treatment with non-steroidal agents, corticosteroids and methotrexate mono-therapy. Previous studies from our centre have shown a positive response to IV TCZ with high rates of remission. TCZ has recently been licensed for administration via the SC route. The aim of this study is to determine how many patients receiving IV TCZ were successfully changed to SC TCZ in the first 6 months after SC TCZ paediatric licensing was obtained and to document any adverse events. Methods Retrospective review of patients with SoJIA or pJIA who were on IV TCZ and changed to S/C TCZ over a 6 month period from October 2018 to April 2019. Patients were identified from an in-house aseptic pharmacy database Clinichemo®. All patients on IV TCZ were identified and cross checked against the Hibernian patient referral system for home SC TCZ administration. Criteria for changing included fulfilling Wallace Criteria for clinical remission and appropriate JIA subtype. Results were analysed using Microsoft Excel. Results 28 patients receiving IV TCZ in October 2018 were identified. 8 were excluded due to either a diagnosis other than SoJIA/pJIA or changing to an alternative biologic. Of the 20 eligible patients, 10 had successfully changed by April 2019. Of those who did not change, 1 is due to change in May 2019, 2 did not wish to change, indicating personal preference as their reason. A total of 7 were deemed unsuitable for medication change due to ongoing active disease. Of the 10 patients who successfully changed, 2 had SoJIA and 8 had pJIA. All were commenced on a dose of 162mg with frequency varying from 1 to 4 weekly as per Summary of Product Characteristics. There was no significant change in ESR, CRP, LFT or WCC demonstrated pre or post change in administration route. No adverse events were reported. 1 patient subsequently changed back to IV administration due to patient preference. Conclusion Our experience to date has been positive, with 50% of patients changed to SC administration within 6 months of approval. A number of other patients have been identified for a change to SC TCZ within the next 6 months. While 1 patient subsequently elected to change back to IV TCZ, to date, no patient has come off SC TCZ due to an increase in disease activity. We hope to follow this study with a patient questionnaire to determine the patient perspective of changing to SC administration of TCZ. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background From our experience, a small cohort of patients with juvenile idiopathic arthritis (JIA) develop irreversible damage to ankle and subtalar joints despite immunosuppression with methotrexate and multiple biologic agents. In this case series, we describe 4 patients with JIA and erosive damage to ankle or subtalar joints who underwent arthrodesis by a single surgeon between 2011-2017 due to longstanding pain and impaired function despite medical and orthotic interventions. We assessed patient satisfaction regarding the procedure in terms of pain and function. There are no previous case series describing arthrodesis of ankle and subtalar joints in JIA. A case series of 8 patients with severe tibiotalar JIA who had arthrodiastasis (joint distraction) reported an improvement in pain and functional scores. Methods In this retrospective case series we reviewed the records of 4 paediatric patients with JIA in Scotland who underwent ankle arthrodesis performed by a single orthopaedic surgeon between 2011 and 2017. Questionnaires were posted out to each patient. These included Manchester-Oxford Foot scores (MOxFQ) and EQ5D scores. Results Time from diagnosis to commencing methotrexate (MTX) was 22 (6-30) months, time from diagnosis to biologic therapy was 5 (2-8) years and time to arthrodesis was 10 (6-14) years (Table 1). Age at surgery was 16 (13-20) years. Prior to surgery all patients reported significant ankle pain and 2 patients needed to wear an ankle-foot orthosis (AFO). Radiological features before surgery in these patients included marked degenerative changes and loss of articular cartilage in the ankle joints on MRI. All patients reported favourable scores in terms of function and pain following ankle or subtalar arthrodesis. Two patients expressed that they wished they had undergone surgery sooner. P12 Table 1: Patient Characteristics Patient  JIA Subtype  Comorbidity  Age at diagnosis of JIA (years)  Systemic Therapy  No of joint injection episodes  Visual Analogue Pain Score Post-Surgery  1  RF –ve Polyarticular  Nil  8  MTX  3    Etanercept  Adalimumab  Abatacept  2  Extended oligoarticular  Uveitis  3  MTX  4  1/10  Adalimumab  3  RF –ve Polyarticular  Nil  6  MTX  4  0/10  Etanercept  4  RF –ve Polyarticular  Trisomy 21, VSD closure, obstructive sleep apnoea  11  MTX  7    Etanercept  Tocilizumab  Patient  JIA Subtype  Comorbidity  Age at diagnosis of JIA (years)  Systemic Therapy  No of joint injection episodes  Visual Analogue Pain Score Post-Surgery  1  RF –ve Polyarticular  Nil  8  MTX  3    Etanercept  Adalimumab  Abatacept  2  Extended oligoarticular  Uveitis  3  MTX  4  1/10  Adalimumab  3  RF –ve Polyarticular  Nil  6  MTX  4  0/10  Etanercept  4  RF –ve Polyarticular  Trisomy 21, VSD closure, obstructive sleep apnoea  11  MTX  7    Etanercept  Tocilizumab  P12 Table 1: Patient Characteristics Patient  JIA Subtype  Comorbidity  Age at diagnosis of JIA (years)  Systemic Therapy  No of joint injection episodes  Visual Analogue Pain Score Post-Surgery  1  RF –ve Polyarticular  Nil  8  MTX  3    Etanercept  Adalimumab  Abatacept  2  Extended oligoarticular  Uveitis  3  MTX  4  1/10  Adalimumab  3  RF –ve Polyarticular  Nil  6  MTX  4  0/10  Etanercept  4  RF –ve Polyarticular  Trisomy 21, VSD closure, obstructive sleep apnoea  11  MTX  7    Etanercept  Tocilizumab  Patient  JIA Subtype  Comorbidity  Age at diagnosis of JIA (years)  Systemic Therapy  No of joint injection episodes  Visual Analogue Pain Score Post-Surgery  1  RF –ve Polyarticular  Nil  8  MTX  3    Etanercept  Adalimumab  Abatacept  2  Extended oligoarticular  Uveitis  3  MTX  4  1/10  Adalimumab  3  RF –ve Polyarticular  Nil  6  MTX  4  0/10  Etanercept  4  RF –ve Polyarticular  Trisomy 21, VSD closure, obstructive sleep apnoea  11  MTX  7    Etanercept  Tocilizumab  Conclusion This case series helps demonstrate patient satisfaction in the small group of patients in our service where irreversible erosive disease caused pain and dysfunction not responsive to medical therapy. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background There is a challenging lack of evidence to inform best practice in the routine clinical care of juvenile idiopathic arthritis (JIA). Wide inter-centre variation in the definition and documentation of clinical data items is a major barrier to improvement. In response, we have developed a consensus agreed standardised core dataset called CAPTURE-JIA (n = 62 data items and a 'data dictionary' including agreed definitions of terms) designed to support routine collection of high-quality clinical data. The feasibility and acceptability of CAPTURE-JIA in clinical practice is not yet known and was the focus of this pilot study. Methods A purposeful sample of six paediatric rheumatology centres across England was invited to collect the CAPTURE-JIA dataset using paper collection forms (n = 20 patients/centre). The dataset was analysed for missing data. Six focus groups (n = 3-10) explored clinicians' views on acceptability and feasibility. Results One hundred and twenty-one patients were recruited over three months. The completeness of the dataset was similar across centres, with minor variations. The majority of data items (eg demographics, dates, ILAR type and examination) were >80% completed. However, 14/62 data items received >40% missing data. (Table 1) Further descriptive analyses highlighted incorrect completion of paper forms. Three themes emerged from the focus groups: problematic data items (missing from >10% forms at > 1 centre), format of clinician data forms and the role of digital data collection. Suggested solutions included minor changes to data item definitions and formatting. There were no refinements to the data items. Development of a digital data collection system was identified by all as essential. Due to a lack of clear consensus, the original CAPTURE forms included a number of ways to record joint count data. This proved confusing and a unanimous decision was taken to collect joint count data on all 83 joints in a tabular format. P10 Table 1: CAPTURE JIA data items with >40% missing data Data item  % forms with data item missing (if item required)  Relevant co-morbidities?  60  Macrophage activation syndrome?  100  Has the ILAR subtype changed?  50  Morning stiffness lasting >15 minutes?  42  History of any form of uveitis?  52  Date started uveitis mediation?  50  Strength of uveitis medication?  83  Counselled prior to new DMARD / biologic?  56  Enrolled in BECS/BCRD if new DMARD / biologic?  48  Joint count (homunculus or table format)  48  Physician assessment of systemic disease activity (VAS)  75  ESR  74  CRP  92  Plasma viscosity  100  Data item  % forms with data item missing (if item required)  Relevant co-morbidities?  60  Macrophage activation syndrome?  100  Has the ILAR subtype changed?  50  Morning stiffness lasting >15 minutes?  42  History of any form of uveitis?  52  Date started uveitis mediation?  50  Strength of uveitis medication?  83  Counselled prior to new DMARD / biologic?  56  Enrolled in BECS/BCRD if new DMARD / biologic?  48  Joint count (homunculus or table format)  48  Physician assessment of systemic disease activity (VAS)  75  ESR  74  CRP  92  Plasma viscosity  100  P10 Table 1: CAPTURE JIA data items with >40% missing data Data item  % forms with data item missing (if item required)  Relevant co-morbidities?  60  Macrophage activation syndrome?  100  Has the ILAR subtype changed?  50  Morning stiffness lasting >15 minutes?  42  History of any form of uveitis?  52  Date started uveitis mediation?  50  Strength of uveitis medication?  83  Counselled prior to new DMARD / biologic?  56  Enrolled in BECS/BCRD if new DMARD / biologic?  48  Joint count (homunculus or table format)  48  Physician assessment of systemic disease activity (VAS)  75  ESR  74  CRP  92  Plasma viscosity  100  Data item  % forms with data item missing (if item required)  Relevant co-morbidities?  60  Macrophage activation syndrome?  100  Has the ILAR subtype changed?  50  Morning stiffness lasting >15 minutes?  42  History of any form of uveitis?  52  Date started uveitis mediation?  50  Strength of uveitis medication?  83  Counselled prior to new DMARD / biologic?  56  Enrolled in BECS/BCRD if new DMARD / biologic?  48  Joint count (homunculus or table format)  48  Physician assessment of systemic disease activity (VAS)  75  ESR  74  CRP  92  Plasma viscosity  100  Conclusion Paper collection of the CAPTURE-JIA data items is feasible and acceptable in the routine clinical setting, but unlikely to be sustainable in the longer term if collected in duplicate with medical notes. A digital tool in the clinical domain, ideally interlocking with local systems, would offer many advantages, including more complete and time-efficient data collection. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Poster presentation Tuesday 8 October Background Over one in four children and young people (CYP) present to paediatric rheumatology services with non-inflammatory conditions. However, there is a lack of evidence-based treatments and clinical guidelines supporting the management of these patients. Our project aims to determine whether CYP diagnosed with different non-inflammatory conditions and their families are satisfied with the service offered by the paediatric rheumatology department at Leeds General Infirmary. In addition, we wanted to assess which interventions they found most helpful and to check if there were differences between children and young people diagnosed with different non-inflammatory conditions. Methods 632 patients seen by Leeds paediatric rheumatology between July 2017 and June 2018 were diagnosed with non-inflammatory conditions; the three most common groups of conditions were symptomatic hypermobility (SH), chronic pain syndrome (CPS) and muscular back pain (MBP). We undertook a patient satisfaction survey, including patients reported assessment of their physical and psycho-social outcomes, focussing on these three groups only. 198 participants (80 SH; 74 CPS; 44 MBP) were invited to the study by sending them a postal questionnaire with self-addressed and stamped envelopes to return them. The questionnaire did not include any identifiable patient information but a different coloured paper was sent to patients with the three different groups of diagnoses to allow comparison of these groups. All had attended the paediatric rheumatology clinic for their initial assessment and had been referred on to appropriate management services as for the treating clinician including physiotherapy, occupational therapy, podiatry/orthotics, pain management, and/or psychology. Results A total of 33 filled questionnaires were received over the next 2 months including 4 (9.09%) from patients diagnosed with MBP, 11 (13.75%) from patients diagnosed with SH and 18 (24.32%) from patients diagnosed with CPS. Mann-Whitney-U calculations were performed to compare groups. The CPS patient group derived less benefit from physical therapies compared with non-CPS patients (U = 35.5, p = 0.0251) and that their reported mental health is worse than non-CPS patients (U = 31.5, p = 0.034). Within the CPS group, patients benefitted more from occupational therapy (U = 13.5, p = 0.01242) and pain management clinic (U = 9.5, p = 0.0226) than podiatry/orthotics services. When asked to rate their overall satisfaction out of 10, the median scores for the SH and MBP groups were 9 and 10 respectively, the median score for the CPS group was 5. Conclusion Our data would suggest that patients suffering with CPS would benefit from a more holistic approach including referrals to a psychologist, occupational therapy and the pain management team. The CPS group seemed most dissatisfied with the services provided by the clinic. The results of the project were fed back to the clinical team and we hope to repeat the survey in future after potential changes suggested by the survey have been implemented. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Poster presentation Tuesday 8 October Background Sjögren's syndrome, a chronic multisystem autoimmune disease is characterised by inflammation of the exocrine glands, principally the salivary and lacrimal glands resulting in xerostomia and xerophthalmia. It can also present with more extensive exocrinopathy as well as extra-glandular, systemic features. Defined as primary SS (pSS) when there is no association with other autoimmune disease, reported incidence and prevalence rates vary. Juvenile-onset pSS is believed to be rare; however it is likely that it is under-recognised and therefore under-diagnosed. To date there have been no studies reporting accurate incidence or prevalence of paediatric pSS (PpSS). Diagnosing pSS can be challenging. Many of the cardinal symptoms are non-specific and no gold standard biomarker of disease exists. Between 1965–2002 eleven diagnostic criteria sets were developed, none of which have gained universal acceptance or been validated for use in a paediatric population. Until recently, the most widely used criteria were those developed by the American-European Consensus Group. It remains well-recognised that international consensus on classification is important for standardisation, particularly in relation to research and monitoring treatment outcomes. With this in mind, the 2016 ACR/EULAR criteria were developed. However, there still remains a paucity of validated classification criteria for diagnosis of juvenile-onset pSS. Paediatric-focused criteria are required as features of pSS in children differ from those observed in adults. Children experience less dryness and more frequently experience systemic symptoms and parotid enlargement. Hence, simply applying adult criteria to a paediatric population may lead to mis- and/or under-diagnosis. The overarching aim of this study is to identify epidemiological, clinical and laboratory characteristics of PpSS in a multi-centre cohort of patients. Using this data our goal is to develop universally accepted classification criteria validated for use in a paediatric population. Going forward, this would enable standardisation of PpSS classification allowing for robust studies into disease pathogenesis, management and prognosis. Methods Inclusion criterion for entry into the UK/Ireland PpSS cohort study and repository is diagnosis of pSS before 18 years. A data collection pack will be sent to authors willing to participate. Information collected will include demographic, clinical and laboratory/histological data at diagnosis and subsequent follow-up appointments. Biological samples including blood, tears, saliva, urine, and glandular and extra-glandular (e.g. renal) tissue will be collected prospectively if available. Outcome measures related to disease activity and damage, as well as patient reported outcomes will also be collected at set time-points. Results No results to report. Conclusion The UK/Ireland PpSS cohort study and repository will capture data on a significant cohort of children with pSS providing a powerful resource to help improve our understanding of this rare disease. Prospective data collection will allow a fuller analysis of poor prognostic features, impact of therapy, damage accrual and variable outcome of PpSS. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Poster presentation Tuesday 8 October Background Musculoskeletal (MSK) problems in children and young people (CYP) are common. The majority will present to healthcare professionals in the community but it can be challenging to identify those with serious disease requiring onward referral. pGALS (paediatric Gait, Arms, Legs and Spine) was developed as a simple, quick MSK clinical assessment to discern abnormal joints, initially within paediatric rheumatology and later targeted at non-specialists. Anecdotally, pGALS can detect joint and functional problems in CYP with other serious conditions, but alone is unlikely to be specific enough. It is unknown whether a pGALSplus assessment is practical. Our aim was to scope the literature about MSK assessments applicable to CYP used in clinical practice, focusing on evidence of validity in the context of diagnosis and assessment of juvenile idiopathic arthritis (JIA), mucopolysaccharidoses (MPS), Muscular dystrophy (MD) and developmental coordination disorder (DCD) to develop an extended pGALS. Methods Scoping review using the Newcastle University Library search tool (which includes several databases) and Google Scholar, and consulting NICE guidance/pathways. Search terms included dyspraxia, paediatric MSK assessment, screening tools, balance, and rheumatology, assessment tools for MD, MPS, and JIA. Studies cited within relevant articles uncovered through searches were also checked. The search was conducted between 1 October and 1 December 2018. Publication date was limited to post-1998, language did not constitute as exclusion criteria unless translation was unavailable. Results 32 journal articles were deemed appropriate, describing specific assessment or screening tools in the context of diagnosis of our target conditions. Within DCD, motor co-ordination test batteries aid diagnosis as part of specialist assessment, but are regarded as too lengthy for the purpose of screening; a questionnaire may be useful as a first-step diagnostic tool, along with an assessment of static balance (found to be significantly worse in children with DCD). In paediatric rheumatology, pGALS is the only validated screening tool to discern normal from abnormal. Other tools to assess health and wellbeing, disability and function are validated in the context of established disease only. For neuromuscular conditions the North Star Ambulatory Assessment is valid, reliable and practical as a functional assessment, and includes activities that are necessary to remain functionally ambulant. With regards to MPS, searches did not reveal specific MSK tests, but evidence suggests that skeletal malformations and joint problems were the most frequently presenting signs. pGALS performs well to identify abnormal joints with restriction within an MPS group. Conclusion This review supports the development of pGALSplus; a toolkit to facilitate identification and assessment of CYP with potentially serious MSK disease. pGALSplus will be targeted at community-based clinicians and will likely include physical examination, questionnaire(s) and appropriate adjuncts. Our group is currently developing pGALSplus, aiming to describe feasibility and acceptability, and develop educational and training resources, aimed at multi-professionals. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Poster presentation Tuesday 8 October Background Haemophagocytic lymphohistiocytosis (HLH)/macrophage activation syndrome (MAS) is a hyperinflammatory syndrome which can complicate sepsis, malignancy or autoimmune/autoinflammatory disease, potentially leading to critical illness and death at all ages. A previous service evaluation of children with ferritin > 10,000 μg/L, which is highly specific for HLH/MAS, reported mortality of 33% and in 44% there was no documented evidence that HLH had been considered by clinicians. The condition may present to generalists, specialists and intensivists and prompt recognition and treatment can be life-saving. A consensus for the management of HLH in older children and adults has not been defined. The aim of this ongoing project is to identify current practice and improve the evidence-base for diagnosis and treatment of HLH. Methods A national cross-specialty group was established in June 2018 including paediatric and adult specialists in rheumatology, haematology, oncology, immunology, infectious diseases, virology, nephrology and intensive care. Clinicians, scientists, pharmacists and data management experts have been incorporated. The group, which numbers over 50 professionals, has met on three occasions to set clinical and research priorities and is supported by Histiocytosis UK, a charity representing patients with HLH. Results The group agreed these priorities: To complete a retrospective service evaluation of adult patients with ferritin > 10,000 μg/L identified over a 3-year period using a standardised proforma established in the previous paediatric cohort [provisionally, this has identified over 200 adults with a mortality rate of 39%] To provide education to clinicians across the UK to improve recognition of HLH [in progress] To develop guidance for investigation of suspected HLH [in progress] To link local multi-specialty teams experienced in managing HLH to create a national network [in progress] To conduct a retrospective, observational study of the use and outcomes of anakinra treatment in HLH To establish a national registry for HLH as part of the UK Histiocytosis Registry (UKHR) using the REDCap online portal for data collection. Patients with definite and probable HLH, including those with MAS secondary to rheumatological conditions, will be eligible for recruitment. Baseline demographic and clinical data, investigations which contribute to the HLH 2004 criteria and H score, and underlying diagnoses will constitute a minimal dataset. Treatments, and responses, and long-term follow-up data will be recorded. In addition to data collection, with the appropriate consents, biological samples will be available for genetic and histopathological analysis and cellular samples for in vitro studies. Conclusion HLH/MAS is an under-recognised condition with high mortality. A national cross-specialty, all-age collaboration has established priorities to improve clinical care and advance research. Development of a registry, with collection of biological samples, will facilitate investigation of disease pathogenesis and help to stratify patients with a view to future clinical trials. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Poster presentation Tuesday 8 October Background The ankle is one of the most commonly affected joints in juvenile idiopathic arthritis (JIA) and ankle joint damage is a known complication. The frequency of ankle damage in modern JIA cohorts is unknown and optimal management pathways are unclear. Magnetic resonance imaging (MRI) is the most sensitive imaging modality for the assessment of joint damage. The aim of this analysis was to investigate the demographics, disease features and management of JIA patients with MRI changes consistent with ankle damage. Methods A single-centre, retrospective study over a four year period was conducted. JIA cases with damage based on MRI features were included in the study. MRI was reviewed by an experienced musculoskeletal radiologist and joint damage was defined as radiological evidence of cartilage loss or joint space narrowing. A standardised electronic pro forma was used to record demographics, disease features and treatments received. Results Fifty one JIA cases had an MRI scan during the study period and 16/51 (31%) had radiological evidence of ankle damage at a median interquartile range (IQR) follow-up of 7.6 (6.9 – 11.0) years; 7/16 (44%) had developed bilateral ankle damage. The median (IQR) duration of symptoms at diagnosis was 2(1.0-10.5) months. The mean age at diagnosis and at detection of ankle damage was 5.2 years and 12.1 years respectively. The most frequent JIA subtype was oligo-articular extended (50%), and 50% were ANA positive. The median (IQR) duration between detection of ankle damage and first episode of ankle synovitis was 5.4 (4.6 – 6.4) years. Ankle synovitis was present at diagnosis in 10/16 (62%). Median (IQR) number of ankle corticosteroid injections before the ankle damage was detected was 3 (2-3), one patient had evidence of damage at diagnosis. Median (IQR) number of any type of ankle imaging done before the ankle damage was detected was 1 (1-3). The median (IQR) time to start methotrexate from diagnosis was 6 (0 - 29.5) months and median (IQR) number of biologic and synthetic DMARDs used at follow-up were 3.5 (2.2 – 4.0). 7/16 (44%) patients had undergone an orthopaedic surgical procedure for their ankle damage. Conclusion Ankle synovitis at presentation and an extended oligo-articular disease course were common in this cohort of children and young people (CYP) with JIA and ankle damage. Ankle damage is an important challenge in JIA; a substantial number of this cohort ultimately required surgical intervention. There is an urgent need to develop a care pathway designed to improve early recognition and management of evolving ankle joint damage in JIA. Our analysis suggests that attention may need to focus on CYP presenting with ankle synovitis, particularly those who develop a poly-articular disease course, may merit high levels of vigilance with be at particularly high risk. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]