Case report - Introduction Bechet’s is a rare chronic inflammatory multisystem disease of unknown cause. It is characterised by recurrent oral ulcerations, genital ulcerations, ocular inflammation and skin involvement. Bechet’s disease is rarely observed during childhood and the clinical picture can be incomplete, thus making the diagnosis challenging. Ocular involvement represents 10—20% of the initial presenting feature. Ocular involvement typically involves anterior and/or posterior uveitis and retinal vasculitis. Severe vision loss can be seen in patients. Prognosis is improving due to enhanced therapeutic options. The use of biologics, particularly anti-TNF drugs, are a positive advance, though more evidence in paediatrics is required. Case report - Case description This young man presented in 2014 at 12 years old to paediatric ophthalmology with a right panuveitis. He was treated with a weaning dose of oral steroids. His right eye became quiet. He reported some oral ulceration, not recurrent, and no genital ulcers. He did not fulfil Bechet’s criteria. Pathergy test and HLA B51 were negative. In 2017 he presented with right macular retinal infiltrate. Vision was reduced to counting fingers. Mycophenolate mofetil (MMF) was started with weaning prednisolone. Despite this, activity was ongoing with inflammation in the anterior chamber and vitreous. Humira was started in August 2017. There was resolution of the inflammation in the right eye but central vision remained poor due to irreversible macular damage. Left eye was normal. Oral steroids were weaned. In 2018, he developed acne and folliculitis type lesions. He reported sore joints and fatigue. He was felt to have probable Behcet’s. Quaternary review by the Bechet’s centre, Liverpool, agreed. A switch from MMF to azathoprine (AZA) was advised. Interferon alpha would have been suitable, but was no longer available. Skin lesions returned when switched to AZA; patient requested switch back to MMF. Over the next 2 years steroids were weaned. MMF and 2-weekly Humira continued. In November 2020 he presented with peripheral retinitis in his left, better-seeing eye, haemorrhaging, with patchy vision. MMF was increased and oral steroids recommenced. In December 2020 visual acuity was hand movements in his right eye and 6/7.5 in his left eye with ongoing inflammation. Adalimumab was increased to weekly. In January 21 there were 3 + of cells in his anterior chamber, extensive haemorrhages and retinal infiltrates in keeping with Behcet’s. He was admitted for pulsed IV methylprednisolone and switched from Humira to infliximab. Eyes are currently quiescent and prednisolone is weaning. Case report - Discussion Bechet’s disease is a multiorgan disease typified by an immune-mediated occlusive vasculitis. The aetiology of Bechet’s remains unclear. HLA-B51 is a major predisposing genetic factor. There is involvement of the innate immune system, propagated by the adaptive immune system. Ophthalmic involvement can be the presenting feature of Bechet’s. Eye disease in Bechet’s is most commonly a non-granulomatous uveitis with necrotising obliterative retinal vasculitis. This can be found in either the anterior or posterior segment or both. Recurrent inflammation in the posterior segment can lead to severe sight loss. Young males typically have the worst ocular prognosis. The treatment of ocular Bechet’s can be challenging as our case emphasises. Treatment follows a step-wise approach. Therapy consists of topical steroid, followed by intravenous or oral steroid. Maintenance therapy of either AZA 2mg/kg/day, MMF (max3 g daily) or ciclosporin max 5mg/kg/day should be commenced. The next step will typically be a TNF inhibitor; in our patient we used adalimumab initially. Infliximab is becoming a popular first choice anti-TNF. Certainly since January 2021 our patient’s eyes have remained quiescent on Infliximab. The alternative to infliximab is interferon alpha but unfortunately this is no longer commercially available. A further treatment that could be offered is rituximab – initially to be given as a one-off cycle. Optimum treatment of ocular Bechet’s involves good multidisciplinary team working between ophthalmology and rheumatology. By early initiation of systemic treatment and regular reviews secondary complications may be avoided/lessened. Case report - Key learning points
Introduction/Background A paediatric rheumatology multi-centre survey was conducted to understand the impact of COVID-19 on services and provide useful learning points that could change future practice. The survey focused on clinicians' views of the pandemic. The aim of this study was to gather the perspectives of young people regarding the results of the initial survey. This was done via a group discussion with members of the national youth advisory panel for adolescent rheumatology – Your Rheum. It is important when evaluating service provision that not only the clinician’s perspective but the patient’s voice is heard. Description/Method The initial survey was sent, in August 2021, to Consultants in all UK paediatric rheumatology centres, to the paediatric rheumatology trainees network and to the JIA topic specific group members via email. 20 staff responded (17 consultants and 3 trainees at registrar level) across the 4 UK nations. Results of the survey showed changes to the frequency of blood monitoring (n = 17), an increase in disease flares (n = 4) with all respondents reporting changes to the type of consultation used with a large increase in the use of virtual consultations. Increases in clinic waiting lists due to capacity issues were also reported (n = 7). 15 respondents reported changes in steroid usage for flare management with more ward based steroid injections performed and an increase in the use of oral steroids due to theatre unavailability. The online discussion group held with ‘Your Rheum’ members consisted of 9 Young People (YP) aged between 12-24 years. The discussion group was facilitated by a research assistant, paediatric rheumatology trainee and a consultant paediatric rheumatologist. A number of statements with Agree/Disagree/Unsure options were provided with an opportunity to vote on each statement. A follow up discussion was held after each statement around the issues raised. The online tool Mentimeter was used to form a word cloud on YP experiences of the pandemic which was followed by breakout rooms to facilitate further small group discussions. Discussion/Results In contrast to the survey results which revealed that clinicians perceived that YP preferred virtual (telephone or video or combination of both) consultations, young people in ‘Your Rheum’ varied in their responses with the majority of comments describing that it depended on the clinical context (if unstable or stable disease) with virtual appointments being preferable when their disease was stable. YP also reported issues with virtual appointments including difficulties with phone signal, not being able to obtain a specific time for appointments (varied time slots), issues for YP with hearing difficulties as well as one YP commenting it is “easier to lie to the doctor and just say everything is going really well”. Negative points raised with face-face appointments included longer waiting times and the time spent travelling to appointments. The majority of young people agreed that changes to the normal pattern of blood monitoring was concerning as this could lead to a possible increase in flare of conditions. Specific comments including accessibility to appointments was difficult during the pandemic with the additional fear of getting COVID-19 if attending the hospital for blood monitoring. YP used a variety of words to explain their experiences of rheumatology services during the pandemic including: ‘rushed’, ‘frustrating’, ‘stressful’, ‘virtual’, ‘chaotic’, ‘poor communication’, ‘inaccessible’ but also ‘COVID safe’. Key learning points/Conclusion Young people with rheumatic diseases offered different insights to the initial clinician survey findings highlighting the importance of considering their opinion as well as that of professionals when considering service provision and evaluation. Flexibility is essential for young people and a hybrid approach with virtual/face to face appointments is key. Overall YP prefer face-face but are willing to have virtual consultations depending on the nature of issues needing addressed and the stability of disease. Experiences of poor communication between YP and health professionals/hospital has always been a theme in their experiences to date but has worsened since COVID-19. YP within this group discussion preferred to have blood monitoring performed at regular frequencies (similar to pre-pandemic) but did have concerns about getting COVID-19 by attending these appointments during the pandemic. It is important that YP are encouraged and reassured about attending appointments whilst ensuring COVID-19 safe precautions.
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