Rachel Tattersall

Diagnosing juvenile idiopathic arthritis

Juvenile Idiopathic Arthritis (JIA), is the commonest cause of chronic arthritis in childhood worldwide, has considerable morbidity and is a common cause of acquired visual loss in children due to the strong association with chronic anterior uveitis. The diagnosis is clinical and confidence in examination of the musculoskeletal system for synovitis is essential. Management of JIA is based on a combination of pharmacological interventions, physical and occupational therapy, and psychosocial support, managed by an experienced multidisciplinary team. The aim of therapy is to reach complete control of the disease, preserve the physical and psychological integrity of the child and to prevent any long-term consequence related to the disease or treatment. There is overwhelming research evidence to support early treatment and aggressive intervention in juvenile idiopathic arthritis. This has developed with the growing awareness that there is a “window of opportunity” to alter the natural history of the disease and the process of inflammation. Early recognition of, and skill in, diagnosis of JIA is therefore essential and reviewed here.

4. A challenging case of refractory BehÇet's disease in an adolescent with sight threatening uveitis

patient.Asignificantlyelevatedtroponinhowever,couldnotbeexplained bya tachyarrhythmiaalone.One hypothesis fordevelopinganacutecoronary syndrome, is the vasodilatation role of TNF in the maintenance of myocardial vascular perfusion through the induction of nitric oxide. It is also capable of inhibiting apoptosis of myocardiocytes and attenuation of cardiac stimulation by the sympathetic nervous system through breceptors. The administration of Infliximab, which is a potent anti-TNF antibody can neutralise both soluble and membrane-bound TNF which can suspend these homeostatic mechanisms, resulting in deprivation of first linedefencesandleadingtocoronaryvasoconstrictionandhypoperfusion. Why patients without prior cardiovascular disease develop such symptomsisstillunclearandmaybeafurtherscopeforresearch. KeyLearning Points:Potentanti-TNFagents have the theoretical ability of causing coronary vasoconstriction and hypoperfusion. Albeit rare, patients presenting with chest pain, during or after the infusion should have the appropriate coronary biochemistry and investigations performed.Youngpeoplewithnoriskofcardiovasculardiseasecandevelop transient acute coronary syndrome in response to potent anti-TNF agents. Doubling the frequency of infusion in attempt to regain control of disease after secondary failure should be done with caution and may increasetherisk. Disclosure: K.M. Achilleos: None. P. Bale: None. A. Shastri: None. N. Puvanachandra:None.K.Armon:None.

Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment

Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which if not promptly treated, can lead rapidly to critical illness and death. HLH is termed macrophage activation syndrome (MAS) when associated with rheumatic disease (where it is best characterized in systemic JIA) and secondary HLH (sHLH) when associated with other triggers including malignancy and infection. MAS/sHLH is rare and coupled with its mimicry of other conditions, is underrecognized. These inherent challenges can lead to diagnostic and management challenges in multiple medical specialties including haematology, infectious diseases, critical care and rheumatology. In this review we highlight the pathogenesis of MAS/sHLH including its underlying triggers, key clinical features and diagnostic challenges, prognostic factors and current treatments in adults.

G258 Diagnosing childhood arthritis: What does it take?

Many young people have an unacceptably long delay in referral to tertiary services often undergoing unnecessary invasive investigations. A validated examination screening tool, pGALS (paediatric gait, arms, legs, spine)1 has been developed but trainees have low confidence examining paediatric joints.2 Local audit data highlights the lack of musculoskeletal examinations in paediatric clerking compared to examinations of other body systems in response to clinical red flags.3 Aims To review the impact of educational clinics on trainees performing pGALS screening examinations and how successful they are at identifying active arthritis (swollen joints) or restricted movement of joints compared to the examination findings of a paediatric rheumatologist performing a pGALS in the same patient. Identify the trend in personal confidence of junior doctors in diagnosing childhood arthritis using the pGALS screening tool with increased exposure to patients with clinical signs of arthritis. A prospective service review of teaching clinics within a tertiary paediatric rheumatology department was performed. Medical students, adult rheumatology trainees and paediatric trainees were invited to attend. Confidence questionnaires were completed before and after clinic attendance. A pGALS proforma was completed by the trainee per patient and sealed in an envelope. A second proforma was completed on the same patient by the consultant before teaching commenced. Data was analysed using sensitivities, specificities and Cohen’s Kappa inter-observer agreement. Adult rheumatology trainees had a higher overall sensitivity for detecting active arthritis (42%) and detecting restriction in movement (45%) compared to other trainee groups (paediatric trainee 34% and 40% respectively, medical student 35% and 33% respectively). Overall specificity was good for active arthritis (90–100%) and restrictive movement (90–100%). Inter-observer agreement was ‘moderate’ for detection of active arthritis and detection of restricted movement ( Table 1 ). A statistically significant (p < 0.05) increase in confidence was seen for the trainee cohort examining the joints of those under 16 years of age, even after only one clinic attendance.Abstract G258 Table 1 The significant improvement in confidence in joint examination is a very positive finding. Performing increased numbers of pGALS examinations increased the sensitivity of picking up abnormal joints. This study has demonstrated the benefit of the pGALS tool as an educational intervention in clinical practice. References Foster, HE, Kay, LJ, Friswell, M, Coady, D and Myers, A. Musculoskeletal Screening Examination (pGALS) for School-Age Children Based on the Adult GALS Screen. Arthritis & Rheumatism (Arthritis Care & Research). 2006;55(5):709–716 Jandial, S, Myers, A, Wise, E and Foster, H Doctors likely to encounter children with musculoskeletal complaints have low confidence in their clinical skills. The Journal of Pediatrics. 2009;154:267–71 Abusrewil, W, Whiteley, A, McMahon, A and Al-Obaidi, M. A, B, C, don’t ever forget the joints. Oral presentation at the Royal College of Paediatrics and Child Health meeting 2013. (Personal communication with Dr W. Abusrewil)

What should adult rheumatology know about paediatric rheumatology

Paediatric and adult rheumatology share many similarities in practice, patterns of disease and treatment and care of patients, but they have some important differences. Paediatric consultations emphasize family, education and developmental concerns as compared with more individualistic consultations in adult services. Young people (YP) with rheumatological disease encounter adult rheumatology during the crucial, tumultuous developmental stage of adolescence (ages 10 19 years) [1]. This may be during a move, ideally through a planned process of transition, from children’s to adult services, or as a firsttime referral. YP therefore experience the interface of paediatric and adult rheumatology at a time of profound personal change and development [2]. The process of transition is important to adult and paediatric rheumatology alike, and rheumatology leads research in this area [3]. However, from the perspective of adult rheumatologists, what is necessary to know about paediatric rheumatology to meet in the middle? Patients with rheumatic disease starting in childhood and adolescence are an important part of adult rheumatologists’ clinical work and a significant proportion require biologic therapy [4]. Therefore adult rheumatologists need to appreciate the features, spectrum and natural history of paediatric rheumatological disease. They also need to be familiar and comfortable with the specific developmental features of adolescence, such as risky behaviours and non-compliance, which impact on health care provision for YP. Adaptations that make services more YP friendly—holding specific YP’s clinics in appropriate environments, adjusting timings to reduce time off school and the innovative use of virtual resources—help engage adolescents [5]. Key in all these aspects are effective, multidisciplinary links between local adult and paediatric rheumatology services in both service delivery and training of staff. In terms of specific disease management, adult rheumatologists need to appreciate paediatric rheumatic disease phenotypes and treatments and how they differ from adult disease. JIA is an umbrella term for several different forms of arthritis, and when JIA exhibits disease activity into adulthood, it should not be seen as seronegative RA. Relabelling risks overtreating the proportion of JIA that will go into remission spontaneously, where treatment should be stopped. Relabelling may also mean patients are lost to follow-up in important registry studies of biologics in JIA. In turn, this contributes to the relative lack of knowledge and invisibility of JIA symptoms, signs and management in adult rheumatology for patients who continue to have active disease into adulthood. The window of opportunity concept in paediatric rheumatology is increasingly recognized, and this imperative to treat inflammatory disease, as well as the range of effective therapies, are very familiar to adult rheumatologists. What may be less familiar to these professionals are some specific features and complications of paediatric rheumatic disease. In JIA, the importance of temporomandibular joint disease, the use of repeated joint injections as monotherapy for oligoarticular disease and the high index of suspicion for recognition and early treatment of macrophage activation syndrome (most typically in systemic JIA) are often underrecognized in adult rheumatology. Similarly, the understanding that presentation of SLE in adolescence is associated with increased morbidity and mortality and the different phenotypes and management strategies of JDM as compared with the adult form are crucial in effective adult rheumatology care. Training programmes for health care professionals often teach either paediatric or adult rheumatology, and wider integration of adolescent rheumatology in each is required. Integration of programmes with each other and the consequent improved knowledge are likely to benefit patient care. In addition to improved training, good links with local paediatric services ensure that YP do not fall into potential knowledge gaps and that individual professionals from multidisciplinary backgrounds in both paediatric and adult rheumatology continually learn from each other. These links are particularly important for adult rheumatologists in understanding issues, such as needle phobia, that YP experience and the potential impact of loss of school time and peer contact during periods of severe illness. Both adult and paediatric rheumatologists routinely see patients with musculoskeletal symptoms and pain that do not have an inflammatory basis. Adult rheumatologists seeing YP need to adopt a paediatric perspective in understanding that such symptoms may be an important marker of psychological distress related to family dynamics or problems in education and may be associated with self-harm. YP and their families have often developed significant trust in and reliance on paediatric services, which can make engagement for YP in adult services problematic. Understanding of such issues requires adult rheumatologists to modify services accordingly. Similarly, paediatric rheumatologists benefit