ObjectiveTo describe current United Kingdom practice in assessment and management of patients with juvenile localised scleroderma (JLS) compared to Paediatric Rheumatology European Society (PRES) scleroderma working party recommendations.MethodsPatients were included if they were diagnosed with JLS and were under the care of paediatric rheumatology between 04/2015–04/2016. Retrospective data was collected in eleven UK centres using a standardised proforma and collated centrally.Results149 patients were included with a median age of 12.5 years. The outcome measures recommended by the PRES scleroderma working party were not utilised widely. The localised scleroderma cutaneous assessment tool was only used in 37.6% of patients. Screening for extracutaneous manifestations did not meet recommendations that patients with head involvement have regular screening for uveitis and baseline magnetic resonance imaging (MRI) brain: only 38.5% of these patients were ever screened for uveitis; 71.2% had a MRI brain.Systemic treatment with disease-modifying anti-rheumatic drugs (DMARDs) or biologics was widely used (96.0%). In keeping with the recommendations, 95.5% of patients were treated with methotrexate as first-line therapy. 82.6% received systemic corticosteroids and 34.2% of patients required two or more DMARDs/biologics, highlighting the significant treatment burden. Second-line treatment was mycophenolate mofetil in 89.5%.ConclusionThere is wide variation in assessment and screening of patients with JLS but a consistent approach to systemic treatment within UK paediatric rheumatology. Improved awareness of PRES recommendations is required to ensure standardised care. As recommendations are based on low level evidence and consensus opinion, further studies are needed to better define outcome measures and treatment regimens for JLS.

No abstract

BackgroundHaemophagocytic lymphohistiocytosis (HLH)/macrophage activation syndrome (MAS) is a hyperinflammatory syndrome potentially leading to critical illness. Early treatment reduces mortality but diagnosis requires a high index of suspicion. Highly elevated ferritin levels (HEF) >10,000 μg/L are highly specific for HLH/MAS [1] and should prompt consideration of hyperinflammation. Diagnostic guidelines for HLH, requiring the presence of ≥5/8 criteria [2], and classification criteria for MAS complicating systemic juvenile idiopathic arthritis (sJIA) have been published [3].ObjectivesTo assess recognition of HLH/MAS in a paediatric population with HEF.MethodsThis retrospective study was conducted at 11 centres under local service evaluation permissions. Biochemistry databases identified patients ≤16 years with serum ferritin >10,000 μg/L during a 3-year period. Each case was assessed against the 2004 HLH criteria and, for patients with sJIA, the 2016 MAS criteria. Due to limited access to some of the laboratory tests, previously-published modified HLH criteria using a threshold of ≥4/5 (excluding tissue haemophagocytosis, decreased natural killer cell function, increased soluble interleukin-2 receptor) were also applied to all patients [4].Results153 patients (55.6% male) were identified. Patient diagnoses included: infections (29.4%), rheumatological (17.0%) and malignancies (17.0%). A diagnosis of HLH/MAS was made by the treating clinical team in 39.9% and considered in a further 16.3%. Using all available data, 30/153 (19.6%) met ≥5/8 criteria and 93.3% of these patients were diagnosed with HLH/MAS by the treating team. 56 (36.6%) met ≥4/5 criteria and 33 (58.9%) of these were diagnosed with HLH/MAS by clinicians. HLH/MAS was not documented as being considered in the differential in 23.2%. Of 23 patients with sJIA, 82.6% met MAS classification criteria and 89.5% of these were diagnosed with MAS by the treating clinicians. Overall mortality was 32.7% (50/153) and was 27.9% (17/61) in patients diagnosed with HLH/MAS during their admission.ConclusionAlthough HEF is highly specific for HLH/MAS, the diagnosis was only made or considered in just over half of paediatric patients with this laboratory result. Increased awareness of this potentially-lethal condition is likely to lead to earlier treatment and reduced mortality.References[1] Allen CE, et al. 2008;50:1227-35.[2] Henter JI, et al. 2007;48:124-31.[3] Ravelli A, et al. 2016;75:481-9.[4] Davì S, et al. 2014;66:2871-80.Disclosure of InterestsEthan Sen: None declared, Beverley Almeida: None declared, Louise Moran: None declared, Charlene Foley: None declared, Nagla Abdelrahman: None declared, Rosie Close: None declared, Ema-Louise Long: None declared, Joshua Bennett: None declared, Jason Palman: None declared, Catriona Anderson: None declared, Kirsty McLellan: None declared, Samundeeswari Deepak: None declared, Kathy Gallagher: None declared, Peter Bale: None declared, Kamran Mahmood: None declared, Clare Pain: None declared, Flora McErlane: None declared, Athim...

ObjectivePaedCbD is a workplace based assessment tool for paediatric trainees that has been designed to assess clinical reasoning and decision-making. Trainees survey highlighted that often trainees felt difficulties in completing the assessments on a regular basis and many WBPAs work place based assessments were completed immediately prior to ARCP. It also appeared that same consultants were carrying out the assessments with junior doctors.MethodologyWe developed a clinic model for carrying out the assessments. all consultants in general paediatrics and all paediatric subspecialties were approached to offer a dedicated 1 PA annual session for this. An electronic booking system was developed with consultant CBD clinic dates and timeslots whereby trainees could self-book specified timeslot. Frequent email reminders were sent to the trainees informing them of the available clinic slots.ResultsWe had a very positive response from both the consultants and trainees. Trainees liked the electronic system as they found it easier than approaching consultants directly for appointments and felt assured the session would happen and necessary forms be completed. Consultants enjoyed these sessions as they had allocated time to spend with the trainees without any disruption and it felt less pressured than in the pre ARCP timeframe. Trainees particularly appreciated the opportunity to discuss subspecialty cases with specialist consultants.ConclusionThis was a successful endeavour. We have run 43 clinics in 1 year period with 13 in first 6 months and 30 in the second 6 months. The clinic model seems to have established well and continuing to expand. Currently all the clinics are fully booked and the clinics are spread throughout the year. General feedback has been most of the trainees are undertaking their assessments in timely fashion in line with their college recommendations. We are hoping to continually improve this model and expand it to do other types of WPBAs as well.

AimTo describe rare and severe complication of Kawasaki’s disease (KD) in a child who was not treated with Immunoglobulins (IVIG).MethodsCase report.ResultsA previously fit and well 4-year-old boy presented with fever, red tongue, rash and cervical adenopathy. He was reviewed in the local hospital and was diagnosed with viral infection. He continued to be febrile and was diagnosed with KD after two weeks, when the skin of his palms and soles started peeling. He was managed conservatively and not given IVIG as the fever had settled. His echocardiogram showed aneurysm of left coronary artery and was commenced on aspirin. At the 3-month follow up with the cardiology team, he was noted to be hypertensive, requiring multiple anti-hypertensives, and was referred to the Paediatric Nephrology team.On assessment, he appeared pale and lethargic and with raised inflammatory markers (ESR 110mm/hr) and nephrotic range proteinuria. His Doppler ultrasound scan showed bilateral renal artery aneurysms. In conjunction with Paediatric Rheumatology team, he was treated with two doses of IVIG with good clinical and biochemical response (ESR 14). His catheter angiogram showed widespread aneurysms affecting all the medium sized abdominal vessels (Figure 1). Because of the extensive involvement, he was treated with methylprednisolone and pulses of IV cyclophosphamide as per the polyarteritis nodosa protocol. He then developed an acute ischaemic cardiac event (Figure 2) and acute kidney injury requiring ventilation and haemodialysis in PICU. He was transferred to quaternary centre for cardiac care, where his coronaries were stented and immunosuppression switched to Infliximab on completion of the Cyclophosphamide regime. He has residual cardiac and renal impairment.Abstract G264 Figure 1Abstract G264 Figure 2ConclusionsThis case highlights the importance of expeditious diagnosis and timely treatment to achieve optimal clinical outcome in KD, which is the commonest cause of acquired heart disease in the west. American Heart Association statement supports the diagnosis of KD even in the absence of full criteria when coronary abnormalities are present. Early referral to tertiary services needs to be considered in an atypical KD with low threshold for treatment with IVIG.