P373 You give me fever! – the autoinflammatory clinic in an irish tertiary paediatric hospital

Introduction Auto-inflammatory syndromes (AIS) are inherited disorders of the innate immune system that lead to pathogenic inflammation. Multiple organ systems can be involved and disease can lead to significant end organ damage. Early diagnosis and treatment may improve outcome;1 Diagnosis is often delayed due to the low incidence of these disorders. An autoinflammatory clinic was established in OLCH Crumlin, Dublin, a tertiary level Paediatric Hospital, in November 2015 to assess patients with known or suspected auto-inflammatory disease within a multi-disciplinary setting. This clinic is attended by immunology, rheumatology and dermatology specialists with multi-disciplinary team input as required. Aim The aim of this study was to survey the diagnosis and management of patients attending this clinic over a 2 and a half year period. Methods A retrospective observational chart review of all patients attending the Autoinflammatory clinic from November 2015 to June 2018. Age of onset, demographic details, diagnosis (if known) and management were documented. Details of any genetic analysis if undertaken were also included. Results A total of 47 patients attended the auto-inflammatory clinic over the identified period. Age of onset at first symptoms ranged from birth to 15 years. A diagnosis of an AIS was made in 57% with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, adenitis) accounting for the majority. An undetermined autoinflammatory syndromes was suspected in 40%. 3% who attended had no symptoms or features supportive of an AIS. Overall, 36% were found to have a confirmed genetic mutation, either benign or pathogenic and 28% had a positive family history of auto-immune or auto-inflammatory syndrome in a 1st degree relative. Colchicine monotherapy (32%) was the first choice of therapy followed by anakinra monotherapy (19%) or a combination of both. All treated patients reported symptom improvement on therapy with the exception of 2. Conclusion This study gives an overview on the spectrum of autoinflammatory disease presenting in Ireland and their current management. The majority of patients attending were appropriately referred to this clinic and had improvement in symptoms following initiation of treatment. Reference Ter Haar NM, Oswald M, Jeyaratnam J, et al. Recommendations for the management of autoinflammatory diseases. Ann Rheum Dis 2015;74:1636–1644.