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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 ).

Introduction We present the case of a 5-year-old boy with episodes of pyrexia of unknown origin associated with polyarthritis, central neuroinflammation and mucocutaneous manifestations. His skin biopsy demonstrated a nodular vasculitis which could be consistent with polyarteritis nodosa. Interestingly though, he had additional atypical rashes, ocular inflammation and evidence of acute and past streptococcal infection and his fever and CRP normalised prior to corticosteroid initiation. Case description A five-year-old British African male presented with five-week history of persistent fever, headaches, joint pain and bilateral eye pain. He was transferred to a tertiary paediatric infectious disease’s unit due to unrelenting fever with no fixed pattern, despite nine days of broad-spectrum intravenous antibiotics. He was diagnosed with neuroinflammation of uncertain aetiology, evidenced by leptomeningeal enhancement and bilateral papillitis on brain MRI. CSF microbiology and autoimmune profiles were negative. There was no evidence of primary malignancy. The fever resolved with pulsed IV methylprednisolone and the child was discharged home on a weaning regime of oral prednisolone. After stopping steroid treatment, he represented with high-grade fever, limping, anterior uveitis and new-onset non tender cutaneous lesions, plaques over eyelids and cheek and annular lesions over arms, legs and buttocks. Repeat MRI of the brain and spine showed resolution of previous neuroinflammation. Blood tests revealed raised ANA, IgG and ASOT titre, and his throat swab was positive for Group A Streptococcus. He completed 10 days of intravenous antibiotics and oral high dose ibuprofen (10 mg/kg TDS). On review by tertiary paediatric rheumatology, he was identified to have pleomorphic skin rash and subcutaneous nodules. Skin and deep tissue biopsy confirmed lobular and septal panniculitis with necrotising vasculitis, consistent with a nodular vasculitis. Treatment with high-dose steroid and mycophenolate mofetil (MMF) was initiated, but his CRP and fever had resolved prior to his first dose. He experienced improvement in systemic symptoms and arthritis; however, skin lesions persisted, with appearance of new lesions and hyperpigmentation, on reduction of steroid dose. His course was complicated by acute varicella infection, requiring oral aciclovir. Further treatment trials included topical tacrolimus and topical corticosteroids for his skin lesions. At one biopsy site, he experienced significant delay in wound healing. The genetic panel for primary immunodeficiency panel (R15) demonstrated no pathological variants. Discussion This case report highlights that, whilst the biopsy may be consistent with nodular vasculitis, such as PAN, the clinical manifestations are not perfectly aligned. Discussion within the rheumatology community would support optimal patient care. Childhood-onset Polyarteritis Nodosa (c-PAN) is a rare systemic vasculitis. Although the aetiology is unknown, associations with infections like group A streptococcus and hepatitis B suggest a postinfectious autoimmune response. There are no clear genetic links to PAN, but PAN-like vasculitis is part of reported monogenic autoinflammatory conditions. Key atypical features in this case include uveitis at presentation, nonspecific central neuroinflammation, unusual cutaneous lesion morphology, improvement of acute phase reactants before immunomodulatory treatment, and delayed biopsy wound healing. While ocular findings in PAN may include episcleritis, scleritis, keratitis, retinal vascular occlusions, and ocular ischemic syndrome, uveitis has not been reported in c-PAN. The patient also showed nonspecific inflammatory changes on initial MRI. Neurological involvement in c-PAN typically includes cerebrovascular accidents, cranial nerve palsies, and mononeuritis multiplex, with meningeal involvement being less common (reported 4% in a study of 69 children). Additionally, the patient experienced delayed wound healing following the biopsy, which is undocumented in c-PAN, but may be related to immune modulating treatment. A positive streptococcal throat swab could suggest an immune reaction as part of bacterial tonsilitis, or Acute Rheumatic Fever. However, he did not meet Jones Criteria, there was no response to prolonged antibiotics and the chronic remitting nature made these less likely. An autoinflammatory disorder, Deficiency of Adenosine Deaminase 2 (DADA2), was also considered but he had no evidence of livedo racemosa, strokes and no CECR1 mutation was identified. Our case has been discussed within a multidisciplinary team and we speculate that it could represent a previously undefined immune sensitivity within the autoimmune, autoinflammatory and immune deficiency spectrum giving an exaggerated response to infection. Key learning points • This case presents a significant diagnostic challenge due to its atypical clinical features and inconclusive extensive investigations, including immunologic testing, tissue biopsy, and genetic analysis. • While the patient’s symptoms improved with high-dose steroids and MMF, the recurrence of symptoms upon tapering steroids underscores the persistent inflammatory nature of the disease. • The detection of group A Streptococcus in the patient’s throat swab suggests a potential postinfectious autoimmune trigger, highlighting the role infections can play in the pathogenesis of vasculitis. The negative results from the R15 Primary Immunodeficiency panel suggest that more advanced genetic and immunologic testing may be beneficial. • We invite discussion on whether additional genetic or immunologic testing could optimise this patient’s care and contribute to the broader understanding of atypical vasculitis syndromes.

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.

Legg-Calve-Perthes’ disease (LCP) is defined as avascular necrosis of the femoral head in a child and may present to a variety of disciplines from general practice to orthopaedics, paediatrics, rheumatology and more. The Stickler syndromes are a group of disorders of type II, IX and XI collagen associated with hip dysplasia, retinal detachment, deafness and cleft palate. The pathogenesis of LCP disease remains an enigma but there have been a small number of cases reporting variants in the gene encoding the α1 chain of type II collagen (COL2A1). Variants in COL2A1 are known to cause type 1 Stickler syndrome (MIM 108300, 609508), which is a connective tissue disorder with a very high risk of childhood blindness, and it is also associated with dysplastic development of the femoral head. It is unclear whether COL2A1 variants make a definitive contribution to both disorders, or whether the two are indistinguishable using current clinical diagnostic techniques. In this paper, we compare the two conditions and present a case series of 19 patients with genetically confirmed type 1 Stickler syndrome presenting with a historic diagnosis of LCP. In contrast to isolated LCP, children with type 1 Stickler syndrome have a very high risk of blindness from giant retinal tear detachment, but this is now largely preventable if a timely diagnosis is made. This paper highlights the potential for avoidable blindness in children presenting to clinicians with features suggestive of LCP disease but with underlying Stickler syndrome and proposes a simple scoring system to assist clinicians.

This case article takes you on a journey starting with a paediatric patient presenting with sudden-onset lower limb paralysis and paraesthesia. Differential diagnoses, investigations and management are discussed as the case progresses, ultimately leading to the underlying cause.

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

Abstract Case report - Introduction Due to dysregulation of their immune system, children with Down syndrome are at increased risk of a number of conditions, including malignancy, infection and autoimmune disorders. This case highlights the importance of the MDT team working in order to fully explore possible diagnoses and reach the correct conclusion. We present a case of a relatively rare eye disorder, initially treated as peri-orbital cellulitis, that required a collaborative team approach to reach the final diagnosis. Case report - Case description A 6-year-old-girl with Down syndrome was referred to the oncology and tertiary ophthalmology teams by her local paediatric team with a history of recurrent right-eye peri-orbital swelling. The impression had been of recurrent peri-orbital cellulitis which was treated with and responsive to antibiotics. However, the most recent presentation was unresponsive to antibiotics and had associated orbital symptoms of proptosis and restricted eye movement. An MRI-head showed a protruding right eye with an enhancing mass centred in the right-superior temporal-extraconal-orbit. The mass was infiltrating the extra-ocular muscles and there was low bone-marrow signal compared to the left-side. The MRI findings were discussed in the oncology MDT-meeting. Appearances were felt to be more in keeping with malignancy, rather than infection. An ophthalmology review confirmed no evidence of optic-nerve compression, so she was discharged on antibiotics with a plan made to admit for biopsy and bone marrow aspirate under general anaesthetic. Histology of the lesion was reported to show a mixed inflammatory-mass, angiocentric with no elements of neoplasia. Oncology-specific stains were negative. BMA was normal. The differential at this point was orbital-pseudo-tumour; if infection and systemic-auto-immune conditions could be excluded. Histochemical-staining of the biopsy excluded fungi, bacteria or mycobacteria. The granulomatous-nature of the infiltrate and the conspicuous presence of eosinophils were consistent with an autoimmune-condition. The little girl was discharged from the oncology team into the care of the ophthalmology and paediatric rheumatology teams for further work-up and management. She was commenced on prednisolone. On rheumatology-review, focussed-history and examination were unremarkable in terms of an underlying systemic-inflammatory condition. A full-blood-work-up was requested. Her pANCA and MPO were mildly raised, as were her inflammatory markers. In view of these results, a CT chest/abdomen angiogram was arranged which was normal. A diagnosis of orbital-pseudotumour was made. There was clinical improvement on prednisolone, so a tapering plan was provided. Case report - Discussion First described by Birch-Hirschfield in 1905, orbital pseudo-tumour, also known as idiopathic orbital inflammatory syndrome (IOIS) is a benign, non-infective, inflammatory condition of the orbit without identifiable local or systemic causes. Post-treatment recurrence occurs in 37% of cases. In this case, as steroids were weaned, there was recurrence of eye symptoms with proptosis and redness of the eye. Repeat bloods were performed, and an MRI-head requested. The repeat ANCA-MPO was negative. Whilst awaiting her MRI, a further clinical review was carried out jointly by paediatric rheumatology and ophthalmology. At this appointment, there was notable proptosis which appeared to be getting worse. The impression was that there was recurrence of the inflammatory swelling, and that the sensible option would be to restart treatment with oral prednisolone. At this point, addition of a steroid-sparing DMARD was discussed. Methotrexate was chosen and commenced. The little girl continued to attend for joint rheumatology and ophthalmology reviews. She continued to have proptosis in the right eye but had excellent ocular motility and good visual-acuity. She was noted to develop a worsening in the lid swelling and possible proptosis with an intercurrent illness, perhaps suggesting that the underlying inflammatory process was still active. The plan was to continue methotrexate for 2 years and perform another orbital MRI before potentially reducing methotrexate. However, in this time the methotrexate dose was reduced due to deranged LFTs. She continued to have “flare ups” with increasing proptosis despite treatment with methotrexate. A repeat MRI after 2 years of treatment showed reduction in the size of the right supra-lateral orbital mass compared to the first scan. However, there was uniform enhancement following gadolinium injection with mild proptosis of the right-globe. The appearances were in keeping with an active granulomatous lesion. The current plan is to try and re-optimise the dose of methotrexate. If not tolerated, an alternative DMARD will need to be considered. Case report - Key learning points The diagnosis of orbital pseudo-tumour is one of exclusion with evaluation directed to exclude neoplasms, infections, and systemic inflammatory disorders. It can manifest as a bilateral condition in 13% and with constitutional symptoms in 40%. Points for discussion Differential of orbital pseudo-tumours in the paediatric population Prognosis of pseudo-tumours Suggested treatment pathway -- Is control to allow normal eye movement enough or should we be treating more aggressively?

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?