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Introduction Joint hypermobility is common in childhood and can be associated with musculoskeletal pain and dysfunction. Current management is delivered by a multidisciplinary team, but evidence of effectiveness is limited. This clinical trial aimed to determine whether a structured multidisciplinary, multisite intervention resulted in improved clinical outcomes compared with standard care. Method A prospective randomised, single centre parallel group trial comparing an 8-week individualised multidisciplinary intervention programme (bespoke physiotherapy and occupational therapy in the clinical, home and school environment) with current standard management (advice, information and therapy referral if deemed necessary). The primary endpoint of the study was between group difference in child reported pain from baseline to 12 months as assessed using the Wong Baker faces pain scale. Secondary endpoints were parent reported pain (100 mm visual analogue scale), parent reported function (child health assessment questionnaire), child reported quality of life (child health utility 9-dimensional assessment), coordination (movement assessment battery for children version 2) and grip strength (handheld dynamometer). Results 119 children aged 5 to 16 years, with symptomatic hypermobility were randomised to receive an individualised multidisciplinary intervention (I) ( n  = 59) or standard management (S) ( n  = 60). Of these, 105 completed follow up at 12 months. No additional significant benefit could be shown from the intervention compared to standard management. However, there was a statistically significant improvement in child and parent reported pain, coordination and grip strength in both groups. The response was independent of the degree of hypermobility. Conclusion This is the first randomised controlled trial to compare a structured multidisciplinary, multisite intervention with standard care in symptomatic childhood hypermobility. For the majority, the provision of education and positive interventions aimed at promoting healthy exercise and self-management was associated with significant benefit without the need for more complex interventions. Trial registration The trial was registered prospectively with the national database at the Clinical Research Network (UKCRN Portfolio 9366). The trial was registered retrospectively with ISRCTN ( ISRCTN86573140 ).

BackgroundChildren with pJIA present with five or more joints affected by pain, swelling and stiffness. Untreated, joint inflammation can lead to irreversible joint damage and disability. Corticosteroids have been used for treatment of JIA since the 1950s. Current clinical practice for treatment of pJIA involves high-dose corticosteroids for a limited period in order to decrease inflammation. The aim is to induce remission whilst systemic treatment, commenced alongside corticosteroids begins to work. No standardised, evidence-based approach currently exists to guide corticosteroid induction regimens in pJIA.ObjectivesDescribe corticosteroid regimens in children newly diagnosed with pJIA.Compare disease activity at diagnosis, with follow-up review after treatment with corticosteroids.MethodsRetrospective chart review of children newly diagnosed with pJIA, January 2019 to December 2020, inclusive. Demographic data collected and steroid regimens documented. Disease activity recorded pre-instigation of corticosteroids, and then at a follow-up appointment on average 5.5 weeks into treatment (range 3–12 weeks). Modified JADAS-27 created using active joint count (AJC), C-reactive protein (CRP) and ESR, as physician and patient/parent global assessment scores were missing from the majority of charts. Total score achievable using modified JADAS-27 (mJADAS-27)=47.ResultsSixteen-children were diagnosed with pJIA between January 2019 and December 2020, (male = 5,15%). Eleven-children (69%) had polyarticular RF-negative JIA (RF-), 3(19%) polyarticular RF-positive JIA (RF+), 1(6%) Psoriatic-JIA (PsA) and 1(6%) HLA-B27 positive enthesitis-related arthritis (ERA). All children were commenced on non-steroidal anti-inflammatory drugs (Naproxen, n=11; Ibuprofen, n=5) and subcutaneous Methotrexate (15mg/m2) alongside corticosteroids.A three-day course of intravenous methylprednisolone (ivMP) was the initial corticosteroid of choice in 12/16(75%) children. Six children were given a dose of 30mg/kg (maximum 1gram), two children 20mg/kg and four 500mg (weight 30.9–44.2 kg; two children were on oral prednisolone (POPred) prior to admission for ivMP; one child had T1DM). Following 3-days of ivMP, all 12 children were commenced on POPred.The children that did not receive ivMP were commenced on POPred at a dose of 0.5–1mg/kg, with an initial weaning plan of 5mg/week. Two of these children had RF+, one had ERA and the other PsA. AJC ranged from 5–12.Starting dose of POPred following 3-days of ivMP ranged from 7.5–40mg, maximum dose 1mg/kg. Weaning instructions varied from 5mg/week (n=6), 2.5mg/week (n=4) or stay on low dose (<0.25mg/kg) until review (n=6).Median mJADAS-27 pre-corticosteroids was 19.4 (5–43.5). Follow-up mJADAS-27 was calculated about 5.5 weeks (3–12) into corticosteroid treatment. Median follow-up mJADAS-27 was 3.5 (0–8). On average, mJADAS-27 improved by 81% (0–100%) following corticosteroids. Of the four children that did not receive ivMP, one child RF- had 100% improvement in mJADAS-27, the other experienced no improvement. The child with PsA experienced 86% improvement; the child with ERA, only a 20% improvement.ConclusionsCorticosteroids lead to improved disease activity in children with pJIA. However, treatment regimens employed vary. Development of a standard operating procedure for corticosteroid induction in pJIA is required. Longitudinal studies would enable evidence-based development of such protocols, and should consider optimal corticosteroid route of administration and dose to achieve maximal benefit, whilst minimising corticosteroid toxicity.

Juvenile idiopathic arthritis (JIA) is an autoimmune disease of childhood requiring treatment with immune modulation therapy. It runs a relapsing and remitting course, with approximately half of affected children continuing with active disease into adult life. Defining clinical remission is challenging, but necessary, as it is critical in determining when potentially toxic therapy can be stopped. We found that preliminary consensus criteria for defining JIA remission are not being used in full by a representative sample of UK pediatric rheumatologists. Extending the period of remission, whilst on synthetic diseasemodifying anti-rheumatic drug (DMARD) medication, beyond 6 months does not seem to reduce the risk of relapse once medication is stopped. However, we found that most clinicians state that they still require at least 1 year in remission before DMARD withdrawal. There is increasing evidence that subclinical biomarkers may help to assess disease activity, and therefore aid clinicians in determining remission. In this review we argue that agreement on remission criteria and optimum timing of DMARD withdrawal is crucial for consistent clinical practice, and further research in this area is needed.

Abstract Case report - Introduction Juvenile idiopathic arthritis (JIA) is a diagnosis of exclusion. In a paediatric rheumatology clinic, children can present with signs and symptoms that don’t quite fit under the umbrella of JIA. When these cases present it is important that we take a thorough history and examination, and investigate as appropriate, with continual assessment to check progress and review response to treatment. This case highlights the importance of this practise, in turn enabling the correct diagnosis to be reached. This allows for more appropriate treatment choices and more specific counselling for child and family with regards to the condition and expected prognosis. Case report - Case description A 7-year-old girl was referred for paediatric rheumatology review with a 3-week history of inability to close her hands properly, and pain and swelling in hands and feet. There had been no preceding illness, trauma, or tick bites. There was no history of associated fever, rash, mouth ulcers or eye symptoms. She complained of central abdominal pain with associated constipation. Her appetite and weight were stable. No significant PMH. FHx brother - JIA. On assessment, systems exam was unremarkable. Her skin was soft with no erythema. It did not appear thickened or shiny, but there was bilateral pitting oedema to just below both knees. On musculoskeletal assessment there was notable dorsal swelling of both hands, with significant restriction of both wrists. There was pain, swelling and restriction of the fingers, elbows, knees, ankles and toes. Her neck and shoulder movements were also restricted. Limited summary of investigation WBC 18.5, Eosinophils 12.77 Albumin 24 (NR 30-50 g/L) ESR 2-7, CRP 6-12 Infection screen / Autoimmune / Scleroderma-Ab-profile / Myositis-Ab-profile NAD X-rays Hands/Feet – NAD Abdominal USS NAD Urine - culture and Alb/Cr ratio NAD Stool culture / parasites / ova – NAD Ophthalmology review NAD An USS was performed of affected joints which showed poly-articular synovitis and tenosynovitis. There was no documented fasciitis. She was treated with three-days IV methylprednisolone (30mg/kg) and discharged on a weaning course of prednisolone. She was reviewed 3-weeks later. She was functioning better but reported on-going abdominal pain and that her arms looked thicker. The skin on her arms appeared thickened. She was unable to make a claw or fully extend fingers. Her wrists were restricted with visible wrist flexor tendons. Her pitting oedema had improved. Her inflammatory markers were normal. She proceeded to have an MRI of her right hand/wrist which confirmed a diagnosis of eosinophilic fasciitis and tenosynovitis. Case report - Discussion Following initial assessment, this 7-year-old girl was found to have significant inflammatory arthritis and tenosynovitis, abdominal pain, eosinophilia and hypo-albuminaemia. However, the clinical picture was not felt to be typical for JIA. The differential diagnosis at the time was that this could be an evolving connective tissue disease; however, the autoimmune screen was negative. Also, initially there were no abnormal skin or fascial features seen on imaging, and no other organ dysfunction or features pointing towards a specific condition. Once infection and risk of malignancy was excluded in consultation with the infectious disease and haematology teams, it was agreed that treatment was required. Pulse methylprednisolone was agreed as an appropriate first choice with a plan to review response on weaning oral steroids. When the little girl returned for review, the clinical picture had progressed to include non-tender, tightness and thickening of the forearms, with ongoing restriction in multiple joints and abdominal pain. In view of the ongoing abdominal pain, eosinophilia, low albumin and rising ALT she was discussed with the gastroenterology team who repeated an abdominal USS and performed an OGD. The USS showed a slightly enlarged spleen. The OGD was macroscopically normal. Microscopically there were some submucosal duodenal foamy macrophages, significance uncertain. The team remained suspicious that this was an eosinophilic driven inflammatory condition with musculoskeletal findings, and so an MRI of the right hand and wrist was requested. This showed an abnormal rim of high signal around the tendons and along the fascial planes of the forearm in both extensor and flexor compartments, consistent with a diagnosis of eosinophilic fasciitis. A skin and muscle biopsy were obtained which confirmed chronic inflammation and fibrosis in the fascia. These features were consistent with the suggested radiological diagnosis of eosinophilic fasciitis. She was commenced on subcutaneous methotrexate. Case report - Key learning points Eosinophilic faciitis (EF), also known as Shulman syndrome after the physician who, in 1974 was the first to report on the disorder in the medical literature, is a rare disorder. It is characterised by inflammatory infiltrate in the fascia consisting of lymphocytes, macrophages and plasma cells, with eosinophils sometimes present. The fascia is thickened 2- to 15- fold; the dermis and epidermis can be unaffected. The condition can precede, coexist or follow localised scleroderma. Clinically it can present as painful swelling with progressive induration and thickening of the skin – “peau d’orange” appearance. In paediatrics, as was the case in this little girl, skin findings are minimal or absent at presentation. Paediatric EF tends to involve extremities, often the hands and feet. There is a paucity of data for paediatric EF. In childhood onset EF there is a higher frequency of joint involvement. However, many can develop persistent cutaneous fibrosis and permanent disability. Over 30% of children also present with visceral involvement, such as mesenteric lymphadenopathy, hepatosplenomegaly and pericardial effusion. Early recognition of the condition and timely initiation of treatment improves likelihood of good response to treatment. Risk factors for persistent fibrosis include extensive disease (3—4 extremities and trunk involvement) and/or younger age at onset. There are no standardised guidelines for treatment of childhood-onset EF. However, given that children have more severe, rapidly progressive articular involvement, the expert view is that initial treatment should include combination therapy i.e., corticosteroids and methotrexate. If this treatment fails, the literature suggests instigation of alternative DMARDS, biologics or JAK inhibitors. Summary learning/discussion points include; EF is Rare, with paucity of paediatric data Comprehensive work up required Early, aggressive treatment important to achieve best outcomes Thorough skin examination important to monitor for localised scleroderma No evidence-based treatment pathway available in paediatric EF

BackgroundWe aimed to provide, through the Uveitis in Childhood National Cohort Study, population-based evidence on incidence, distribution and disease characteristics for childhood onset non-infectious uveitis.MethodsEligible children and young people (<18 years) were those newly diagnosed with non-infectious uveitis between 1 March 2020 and 28 February 2023. Cases were identified and recruited through passive surveillance across a multicentre network. Descriptive analysis of demographic, socioeconomic and clinical characteristics at diagnosis is reported alongside incidence rates, relative rates by region and sociodemographic patterning.Results468 cases were identified, providing a minimal national disease incidence of 1.89/100 000 (95% CI 1.72 to 2.07). Among the 255 children recruited, anterior uveitis was predominant (76.9%) and 65% of cases were bilateral. Peak incidence was at 11–15 years. Children resident in deprived areas and those from non-White ethnic backgrounds were over-represented (28% and 31% of the cohort). One in seven children (15%) had a diagnosis of juvenile idiopathic arthritis (JIA), and 5% had tubulointerstitial nephritis. Although bilaterally poor vision was uncommon (16.8%), 44.3% had lost some vision in at least one eye.ConclusionsThere is a need to reconsider how best to deliver paediatric rheumatological and eye care that meets the needs of young people, as well as young children, with uveitis. The predominance of non-JIA-related uveitis calls for a shift in focus. There appears to be socioeconomic drivers of disease risk, which are worthy of future exploration and which have implications on the delivery of care for this chronic and blinding disease.

Abstract Case report - Introduction The differential diagnosis of paediatric uveitis is extensive. Classification starts by determining infectious versus non-infectious causes, anatomic location and associated intra-ocular and extra-ocular features. A relatively common referral to Paediatric Rheumatology from our Ophthalmology colleagues is of a child with a diagnosis of uveitis. This case highlights the importance and benefits of multidisciplinary team working across our regional network when caring for children with complex and rare conditions. Case report - Case description An 8-year-old-girl was referred by her local ophthalmology team to the paediatric rheumatology clinic with a diagnosis of pars-planitis. She had presented to them with blurred-vision and eye-“floaters”. On review, the girl reported that she had a 4-month-history of headaches and blurred-vision, associated with dizziness. She denied any eye-pain. Corresponding to the onset of her symptoms, she had suffered with Chickenpox. Her mother felt that she had \"not been right since then\". She noted that she was generally quieter and more fatigued than normal. Over the course of the 4 months, she was noted to have become clumsier and was bumping into things on a regular basis. She reported increasing visual difficulties in her right eye and initially attended for an optician review. The optician was concerned and referred for urgent ophthalmology opinion. She was diagnosed with bilateral pars-planitis, commenced on oral prednisolone and referred for paediatric rheumatology and tertiary ophthalmology assessment. The headaches improved following commencement on steroids. Positive findings on systems review included occasional oral ulcers, 1—2 times-per-month. They started about 1-month prior to the onset of her chickenpox and continued for the following 3—4 months. She denied any history of genital-ulcers or skin-rash. She had new-onset muscle soreness and tiredness after activity. She also described non-specific abdominal pain since having chickenpox, but no associated change in bowel habit. Examination revealed normal skin, hair, nail and joint examination. She had a soft systolic murmur (echocardiogram normal). She had RUQ tenderness on abdominal palpation (abdominal-USS – spleen upper limit of normal. Nil else). Relevant blood and stool samples were sent as part of a uveitis work-up panel. The paediatric ophthalmologist found right-intermediate uveitis with vitreitis and peripheral retinal changes in keeping with a peripheral exudative detachment. Similar changes were seen in the peripheral retina of the left eye. The impression was of bilateral pan-uveitis with retinal involvement. A subsequent oral fluorescein angiogram showed a widespread retinal vasculitis with some occlusive changes in the right-eye, and a tuft of retinal vascular leakage at the 5-o'clock position in the left eye. Case report - Discussion The differential diagnosis for paediatric retinal vasculitis is broad. It includes collagen vascular disorders, Behçet's disease, Eales’ disease, post viral or post vaccination, acquired toxoplasmosis, multiple sclerosis, systemic immunosuppression and Henoch-Schönlein purpura. The ANA, ENAs, dsDNA, ANCA, ACE, Toxoplasma and Lyme serology were all negative for this little girl. Her inflammatory markers were also normal. She was not on any regular medications prior to her illness and had not had any recent vaccinations. She had no significant past medical or family history of note. However, the onset of her symptoms did correspond to her having chickenpox. She was subsequently found to be positive for HLA B51. In view of the ophthalmology findings and positive HLA B51, the little girl was admitted for an urgent MRI/MRI to exclude neuro- Behçets. This was reported as normal. She was treated with a three-day pulse of IV methylprednisolone and discharged on oral prednisolone 10mg OD (weight 33.4kg). At present Bechet’s retinal vasculitis remains high in the list of differentials for this little girl; however, currently she does not strictly meet the diagnostic criteria for Bechet’s disease. She has been commenced on a steroid sparing agent, azathioprine. Her follow-up plan is to be reviewed in the joint paediatric rheumatology and ophthalmology clinic in 1 months’ time. Case report - Key learning points Retinal vasculitis may occur secondary to a systemic disease or an infectious agent, or as an isolated retinal aetiology. Given that the differentials are vast, a detailed history and examination are important to identify signs and symptoms of systemic disease. Appropriate investigations should be chosen to help narrow the differentials and ensure pathology that could lead to significant morbidity and mortality is not missed. With a case such as this, close collaboration between the paediatric ophthalmologist and rheumatologist is paramount to ensure the best outcome for the patient. With regards to treatment, small case series have described a refractory nature of retinal vasculitis in paediatric patients. One study report that almost 80% of patients with paediatric idiopathic uveitis show manifestations of retinal vasculitis, which is associated with a lower probability of inflammation control resulting in a worse visual prognosis. Points for discussion Thoughts on differential diagnosis? Thoughts on appropriate treatment when no definitive cause identified. Should we follow the National Behcet’s Disease pathway? Expected prognosis for paediatric retinal vasculitis?