The ANCA associated vasculitides (AAVs) affect a range of internal organs including ear nose and throat, respiratory tract, kidneys, skin and nervous system. They include granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA). The AAVs are treated with high dose glucocorticoids, immunosuppressants, and targeted biological medications. Since the 1990s classification criteria for the AAVs have been based on clinical features, laboratory tests and basic imaging; an initiative to update the classification criteria incorporating newer tests, for example, anti-neutrophil cytoplasmic antibodies (ANCA) and novel imaging techniques will be published this year. There is also evidence for classification of patients based on ANCA subtype; those with anti-proteinase 3 antibodies (PR3) or anti-myeloperoxidase antibodies (MPO) have differences in response to treatment and clinical outcomes. An update is described within this review. The pathogenesis of AAV involves necrotizing inflammation of small to medium blood vessels involving multiple immunological pathways. We present an update on emerging evidence related to auto-antibodies, complement and lymphocyte pathways. This review describes emerging treatment regimens, including evidence for plasma exchange in severe disease and the inhibitor of the complement C5a receptor (C5aR) inhibitor, Avacopan. Lastly, patient reported outcomes are key secondary outcomes in randomised controlled trials and increasingly clinical practice, we report development in disease specific and glucocorticoid-specific PROs.

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Purpose of reviewThis review paper evaluates the use of patient reported outcome (PROs) in systemic vasculitis and the increasing incorporation of these measures in the evaluation of clinical outcomes and healthcare provision. Recent findingsGeneric PROs such as the SF-12, SF-36, EQ-5D have been used to evaluate health-related quality of life (HRQOL) across the spectrum of vasculitis; including giant cell arteritis, antineutrophil cytoplasmic antibody (ANCA)-related vasculitis and immunoglobulin A vasculitis (IgA) vasculitis. More recently disease-specific PROs have been developed including the associated vasculitis (AAV)-PRO and GCA-PRO, whilst further work is ongoing including a Steroid-PRO.

Kikuchi-Fujimoto disease is a rare, benign cause of necrotising lymphadenitis often presenting with fever. We describe a case of a 17-year-old boy with non-verbal autism presenting to our intensive care unit with prolonged fever of unknown cause. This case highlights the role of the intensive care unit in cases of diagnostic dilemma. The critical care community should be aware of Kikuchi-Fujimoto disease as although it is usually benign, it can rarely lead to acute airway compromise.

Background/Aims Over the last 20 years, innovation in digital health technology have been propelling health systems forwards. With the advent of the electronic health record, digital prescribing and artificial intelligence and machine learning comes new opportunities. The use of voice recognition systems in the transcription of rheumatology clinics has the potential for cost and time effectiveness which is ever important within the NHS. Here we review the use, user-interface acceptability and potential cost savings. Methods In speech recognition software the speech is converted to a sequence of words in written text. This is not a new technology and in particular has been widely used within radiology departments across the UK. Rheumatology is largely a clinic-based specialty, with high output of clinic letters. Speech recognition software removes the need for transcription-based services. As a new technology we have reviewed its use within the rheumatology, dermatology, endocrinology, renal and paediatric departments at a large district general hospital. Results 143 staff members have been provided with a license and on average 64% are regularly using the available software. Over 3 months an average of 14,843 minutes per month of dictation has been completed. It has been estimated that over 12 months’ use there has been £100,000 savings through administerial time saved. The average turnaround time for clinic letters from dictation to delivery (electronically) to GPs has improved from approximately 1-2 weeks (but delays up to 4 weeks seen) to an approximately 24-36 hours. Preliminary data from users of this software suggest that compared to traditional dictation devices: 33% found it ‘much better’, 33% ‘better’ and 33% ‘the same’. 67% of participants found it ‘easy, or very easy’ to use whilst 16% found it ‘difficult’. 50% of participants found it ‘usually’ saved time, 33% ‘sometimes’ and 16% ‘rarely’ saved time. Conclusion This preliminary data suggest potential time and cost savings with largely positive feedback from users. Further work is needed to assess potential patient safety issues including errors and inaccuracies compared to traditional dictation means. We aim to complete this work prior to presentation of this abstract should it be accepted. Disclosure H. Crawshaw: None. S. Jamal: None. R. Andev: None.