P29 A case of paediatric ocular Behcet’s

• Published in: Rheumatology advances in practice 2021-10, Vol.5 (Supplement_1)
• Main Authors: McKenna, Dearbhla, McLaughlin, Diarmuid, McLoone, Eibhlin, Campbell, Cathy, Rooney, Madeline, McAvoy, Clara
• Format: Article
• Language: English
• ISSN: 2514-1775; 2514-1775
• DOI: 10.1093/rap/rkab068.028

Abstract 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 in