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OBJECTIVE: To compare the responsiveness of the British Isles Lupus Assessment Group 2004 index (BILAG-2004) and the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) disease activity indices and to determine whether there was any added value in combining BILAG-2004, BILAG-2004 system tally (BST), or simplified BST (sBST) with SLEDAI-2K. METHODS: This was a multicenter longitudinal study of SLE patients. Data were collected on BILAG-2004, SLEDAI-2K, and therapy on consecutive assessments in routine practice. The external responsiveness of the indices was assessed by determining the relationship between change in disease activity and change in therapy between 2 consecutive visits. Comparison of indices and their derivatives was performed by assessing the main effects of the indices using logistic regression. Receiver operating characteristic curves analysis was used to describe the performance of these indices individually and in various combinations, and comparisons of area under the curve were performed. RESULTS: There were 1,414 observations from 347 patients. Both BILAG-2004 and SLEDAI-2K maintained an independent relationship with change in therapy when compared. There was some improvement in responsiveness when continuous SLEDAI-2K variables (change in score and score of previous visit) were combined with BILAG-2004 system scores. Dichotomization of BILAG-2004 or SLEDAI-2K resulted in poorer performance. BST and sBST had similar responsiveness as the combination of SLEDAI-2K variables and BILAG-2004 system scores. There was little benefit in combining SLEDAI-2K with BST or sBST. CONCLUSION: The BILAG-2004 index had comparable responsiveness to SLEDAI-2K. There was some benefit in combining both indices. Dichotomization of BILAG-2004 and SLEDAI-2K leads to suboptimal performance. BST and sBST performed well on their own; sBST is recommended for its simplicity and clinical meaningfulness.

Objective To determine whether BILAG-2004 index is associated with the development of damage in a cohort of SLE patients. Mortality and development of damage were examined. Methods This was a multicentre longitudinal study. Patients were recruited within 12 months of achieving fourth ACR classification criterion for SLE. Data were collected on disease activity, damage, SLE-specific drug exposure, cardiovascular risk factors, antiphospholipid syndrome status and death at every visit. This study ran from 1 January 2005 to 31 December 2017. Descriptive statistics were used to analyse mortality and development of new damage. Poisson regression was used to examine potential explanatory variables for development of new damage. Results A total of 273 SLE patients were recruited with total follow-up of 1767 patient-years (median 73.4 months). There were 6348 assessments with disease activity scores available for analysis. During follow-up, 13 deaths and 114 new damage items (in 83 patients) occurred. The incidence rate for development of damage was higher in the first 3 years before stabilizing at a lower rate. Overall rate for damage accrual was 61.1 per 1000 person-years (95% CI: 50.6, 73.8). Analysis showed that active disease scores according to BILAG-2004 index (systems scores of A or B, counts of systems with A and BILAG-2004 numerical score) were associated with development of new damage. Low disease activity (LDA) states [BILAG-2004 LDA and BILAG Systems Tally (BST) persistent LDA] were inversely associated with development of damage. Conclusions BILAG-2004 index is associated with new damage. BILAG-2004 LDA and BST persistent LDA can be considered as treatment targets.

Objective GCA is the commonest primary systemic vasculitis in adults, with significant health economic costs and societal burden. There is wide variation in access to secondary care GCA services, with 34% of hospitals in England not having any formal clinical pathway. Quality standards provide levers for change to improve services. Methods The multidisciplinary steering committee were asked to anonymously put forward up to five aspects of service essential for best practice. Responses were qualitatively analysed to identify common themes, subsequently condensed into domain headings, and ranked in order of importance. Quality standards and metrics for each domain were drafted, requiring a minimum 75% agreement. Results 13 themes were identified from the initial suggestions. Nine quality standards with auditable metrics were developed from the top 10 themes. Patient Access, glucocorticoid use, pathways, ultrasonography, temporal artery biopsy, PET scan access, rheumatology/ophthalmology expertise, education, multidisciplinary working have all been covered in these quality standards. Access to care is a strand that has run through each of the developed standards. An audit tool was developed as part of this exercise. Conclusion These are the first consensus auditable quality standards developed by clinicians from rheumatology and ophthalmology, nursing representatives and involvement of a patient charity. We hope that these standards will be adopted by commissioning bodies to provide levers for change from the improvement of patient care of individuals with GCA.

Objectives Although the painful and disabling features of early diffuse cutaneous SSc (dcSSc) have an inflammatory basis and could respond to corticosteroids, corticosteroids are a risk factor for scleroderma renal crisis. Whether or not they should be prescribed is therefore highly contentious. Our aim was to examine safety and efficacy of moderate-dose prednisolone in early dcSSc. Methods PRedSS set out as a Phase II, multicentre, double-blind randomized controlled trial, converted to open-label during the Covid-19 pandemic. Patients were randomized to receive either prednisolone (∼0.3 mg/kg) or matching placebo (or no treatment during open-label) for 6 months. Co-primary endpoints were the HAQ Disability Index (HAQ-DI) and modified Rodnan skin score (mRSS) at 3 months. Over 20 secondary endpoints included patient reported outcome measures reflecting pain, itch, fatigue, anxiety and depression, and helplessness. Target recruitment was 72 patients. Results Thirty-five patients were randomized (17 prednisolone, 18 placebo/control). The adjusted mean difference between treatment groups at 3 months in HAQ-DI score was −0.10 (97.5% CI: −0.29, 0.10), P = 0.254, and in mRSS −3.90 (97.5% CI: −8.83, 1.03), P = 0.070, both favouring prednisolone but not significantly. Patients in the prednisolone group experienced significantly less pain (P = 0.027), anxiety (P = 0.018) and helplessness (P = 0.040) than control patients at 3 months. There were no renal crises, but sample size was small. Conclusion PRedSS was terminated early primarily due to the Covid-19 pandemic, and so was underpowered. Therefore, interpretation must be cautious and results considered inconclusive, indicating the need for a further randomized trial. Trial registration ClinicalTrials.gov, https://clinicaltrials.gov, NCT03708718.

This guideline will provide a practical roadmap for management of SSc that builds upon the previous treatment guideline to incorporate advances in evidence-based treatment and increased knowledge about assessment, classification and management. General approaches to management as well as treatment of specific complications will be covered, including lung, cardiac, renal and gastrointestinal tract disease, as well as RP, digital vasculopathy, skin manifestations, calcinosis and impact on quality of life. It will include guidance related to emerging approved therapies for interstitial lung disease and account for National Health Service England prescribing policies and national guidance relevant to SSc. The guideline will be developed using the methods and processes outlined in Creating Clinical Guidelines: Our Protocol. This development process to produce guidance, advice and recommendations for practice has National Institute for Health and Care Excellence accreditation.

Background/Aims Rheumatologists prescribe many hospital-only medications (also known as red category prescriptions) including biologicals, chemotherapy such as cyclophosphamide, subcutaneous methotrexate and bosentan. The use of these medicines should be clearly communicated to primary care who should record this information in the patient’s electronic record to reduce the risk of a primary care clinician prescribing an incompatible medication and thus avoiding any potentially severe adverse events. It is also important to provide a complete list of medication when a patient is admitted to hospital acutely or referred to another department. Reflecting on a recent fatal incident, where a patient receiving subcutaneous methotrexate was prescribed trimethoprim by her GP and developed neutropaenic sepsis, our team was prompted to assess whether hospital-only prescriptions in our rheumatology cohort of patients are being flagged as a red drug in the GP system. Our objective was to determine if non-GP prescribed red drugs/hospital-only prescribed medications are being recorded in the patient’s primary care medication record. Methods We randomly selected 100 patients who attended the rheumatology day case unit from March 2021 to June 2021. We checked the newly available regional electronic patient record which contains information on medications listed on the primary care record, to see if the red drug was recorded in this record. We also recorded a patient’s age, gender, rheumatological diagnosis and the name of the hospital-only medication. Results Alarmingly, 50% of hospital-only medications were unflagged in the GP record system raising concerns about patient safety. 75% of the patients evaluated were female and their mean age was 56 (standard deviation 16.3). 61% were diagnosed with an inflammatory arthritis, of which rheumatoid arthritis was the commonest. Patients with CTD and vasculitis accounted for 39% of the population. 11% were receiving chemotherapy, 92% biologics and 12% subcutaneous methotrexate. A biological therapy was missed out more frequently compared with other categories. Conclusion A very high number of GP records did not have the hospital-only/red flag drugs recorded. This is a potential significant risk. The risk needs to be reduced by better communication between secondary care and primary care. Secondary care clinicians working in our trust can now check that the hospital only medication is correctly recorded on the GP prescribing system. If it is not recorded, then it needs to be highlighted to the GP practice in discharge letters and clinic letters. We hypothesise that this incomplete recording is not unique to our trust but a wider problem which needs to be addressed urgently to avoid preventable deaths. We propose that organisations ensure information about medicines prescribed elsewhere is reviewed on the GP clinical system at least annually, even if there are no changes to the medication. We as clinicians need to put patient safety first as an integral part of professionalism. Disclosure L. Rajagopala: None. A. Jain: None. S.Z. Chowdhury: None. D.A.E. Gamage: None. Y.A.A. Ali: None. B. Griffiths: None.

Background/Aims The objective of this project is to map services essential to delivering high quality care in giant cell arteritis (GCA) across England, identifying gaps in provision and thereby help to remove inequalities. To do this however, there must first be agreement on what these best practice services and standards are. Methods A steering committee was formed comprising 18 expert representatives from the 13 clinical regions in England, including rheumatology, ophthalmology, allied health professional and patient representation. A modified Delphi process was commenced with each member initially providing five aspects of service they felt were essential for best practice GCA care. From the 90 answers, common themes were identified by creation of a word cloud and then condensed into domains of practice. These domains were then ranked by each member in order of perceived importance. The top 10 domains taken forward for further review were clinical pathways, patient access, Rheumatology involvement, Ophthalmology involvement, ultrasonography provision, temporal artery biopsy provision, PET-CT scan provision, glucocorticoid treatment, patient education and multi-disciplinary team working. Domains identified as separate areas but not quite making it into the top 10 were Tocilizumab provision, audit and governance and research. With the latter two in particular, it was felt these are overarching principles which should run through all aspects of clinical work. Group consultation was undertaken to discuss the relevant aspects, and from this, three quality metrics and one summary statement were devised for each domain. Rheumatology and Ophthalmology provision were amalgamated, as it was felt these were equally as important, with similar requirements. On the first pass of voting all except ‘patient access’ achieved over 75% agreement amongst the steering committee members. After group consultation and amendment, ‘patient access’ also achieved the minimum 75% agreement cut-off. The final statements can be seen in the table below. Results: Conclusion By devising specific quality metrics in addition to the recommendation statements above, it is envisaged these standards can be easily used as an audit tool to identify gaps and development needs in GCA services. Disclosure F.L. Coath: None. M. Bukhari: None. G. Ducker: None. B. Griffiths: None. S. Hamdulay: None. M. Hingorani: None. C. Horsbrugh: None. C. Jones: None. P. Lanyon: None. S. Mackie: None. S. Mollan: None. J. Mooney: None. J. Nair: None. E. O’Sullivan: None. A. Patil: None. J. Robson: None. V. Saravanan: None. M. Whitlock: None. C. Mukhtyar: None.

Objectives: This study aimed to demonstrate how to estimate the value of health gain after patients with a multisystem disease achieve a condition-specific composite response endpoint. Methods: Data from patients treated in routine practice with an exemplar multisystem disease (systemic lupus erythematosus) were extracted from a national register (British Isles Lupus Assessment Group Biologics Register). Two bespoke composite response endpoints (Major Clinical Response and Improvement) were developed in advance of this study. Difference-in-differences regression compared health utility values (3-level version of EQ-5D; UK tariff) over 6 months for responders and nonresponders. Bootstrapped regression estimated the incremental quality-adjusted life-years (QALYs), probability of QALY gain after achieving the response criteria, and population monetary benefit of response. Results: Within the sample (n = 171), 18.2% achieved Major Clinical Response and 49.1% achieved Improvement at 6 months. Incremental health utility values were 0.0923 for Major Clinical Response and 0.0454 for Improvement. Expected incremental QALY gain at 6 months was 0.020 for Major Clinical Response and 0.012 for Improvement. Probability of QALY gain after achieving the response criteria was 77.6% for Major Clinical Response and 72.7% for Improvement. Population monetary benefit of response was £1 106 458 for Major Clinical Response and £649 134 for Improvement. Conclusions: Bespoke composite response endpoints are becoming more common to measure treatment response for multisystem diseases in trials and observational studies. Health technology assessment agencies face a growing challenge to establish whether these endpoints correspond with improved health gain. Health utility values can generate this evidence to enhance the usefulness of composite response endpoints for health technology assessment, decision making, and economic evaluation.

Objectives We aimed to estimate what proportion of people with SLE attending UK rheumatology clinics would be categorized as being at high risk from coronavirus disease 2019 (COVID-19) and therefore asked to shield, and explore what implications this has for rheumatology clinical practice. Methods We used data from the British Society for Rheumatology multicentre audit of SLE, which included a large, representative cross-sectional sample of patients attending UK Rheumatology clinics with SLE. We calculated who would receive shielding advice using the British Society for Rheumatology’s risk stratification guidance and accompanying scoring grid, and assessed whether ethnicity and history of nephritis were over-represented in the shielding group. Results The audit included 1003 patients from 51 centres across all 4 nations of the UK. Overall 344 (34.3%) patients had a shielding score ≥3 and would have been advised to shield. People with previous or current LN were 2.6 (1.9–3.4) times more likely to be in the shielding group than people with no previous LN (P Conclusion Our study demonstrates the large number of people with SLE who are likely to be shielding. Implications for clinical practice include considering communication across language and cultural differences, and ways to conduct renal assessment including urinalysis, during telephone and video consultations for patients who are shielding.

Summary Background Systemic sclerosis-associated interstitial lung disease (ILD) carries a high mortality risk; expert guidance is required to aid early recognition and treatment. We aimed to develop the first expert consensus and define an algorithm for the identification and management of the condition through application of well established methods. Methods Evidence-based consensus statements for systemic sclerosis-associated ILD management were established for six domains (ie, risk factors, screening, diagnosis and severity assessment, treatment initiation and options, disease progression, and treatment escalation) using a modified Delphi process based on a systematic literature analysis. A panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in systemic sclerosis-associated ILD participated in three rounds of online surveys, a face-to-face discussion, and a WebEx meeting, followed by two supplemental Delphi rounds, to establish consensus and define a management algorithm. Consensus was considered achieved if at least 80% of panellists indicated agreement or disagreement. Findings Between July 1, 2018, and Aug 27, 2019, consensus agreement was reached for 52 primary statements and six supplemental statements across six domains of management, and an algorithm was defined for clinical practice use. The agreed statements most important for clinical use included: all patients with systemic sclerosis should be screened for systemic sclerosis-associated ILD using high-resolution CT; high-resolution CT is the primary tool for diagnosing ILD in systemic sclerosis; pulmonary function tests support screening and diagnosis; systemic sclerosis-associated ILD severity should be measured with more than one indicator; it is appropriate to treat all severe cases; no pharmacological treatment is an option for some patients; follow-up assessments enable identification of disease progression; progression pace, alongside disease severity, drives decisions to escalate treatment. Interpretation Through a robust modified Delphi process developed by a diverse panel of experts, the first evidence-based consensus statements were established on guidance for the identification and medical management of systemic sclerosis-associated ILD. Funding An unrestricted grant from Boehringer Ingelheim International.

Background and study aimImmunoglobulin G4-related disease (IgG4-RD) is a rare immune mediated fibroinflammatory condition. Pancreaticobiliary (PB) and head and neck (HN) are two of the most commonly involved anatomical sites. It has been postulated that PB IgG4-RD and HN IgG4-RD have distinct clinical phenotypes. Whether the optimum treatment regimen or response to therapy differs between them is unknown. We aimed to assess differences between PB and HN IgG4-RD in a cohort of IgG4 disease managed by an IgG4-RD multispecialty team.MethodsWe performed a retrospective study of a prospectively maintained multidisciplinary IgG4-RD database to identify patients diagnosed with PB and HN IgG4-RD (based on initial presentation) between 2005 and 2019. The electronic patient records were reviewed. Use of immunosuppressive agents and clinical course was analysed.Results60 patients with PB IgG4-RD and 14 with HN IgG4-RD were included in the study. PB IgG4-RD was associated with older age at diagnosis 64 versus 51 years (pConclusionThese findings support the contention that PB IgG4-RD and HN IgG4-RD have different clinical profiles and represent distinct subtypes of IgG4-RD.

Background/Aims Severe Raynaud’s phenomenon (RP) can lead to digital ulcers (DU), ischaemia, infection and gangrene. In 2015, NHS England published a commissioning policy enabling the use of bosentan for digital ulceration in SSc in patients refractory to intravenous 6-8 weekly prostanoid in combination with sildenafil following standard therapy (including calcium channel blockers (CCB), ACE inhibitors, losartan and fluoxetine). Bosentan is licensed to prevent new DUs in SSc. Specialist MDT ratification and Blueteq registration is required. RCTs showed bosentan reduced the formation of new DU by 30-50% in at risk individuals. It is a well-tolerated drug. It is now off-patent so its cost has reduced from £22,000 to £650 per year. Aim To audit current departmental practice in patients receiving prostanoid (epoprostenol) for severe RP from any cause and check adherence to the patient pathway for treatment escalation prior to prostanoid therapy. To determine approximate costs of alternative therapeutic approaches. Methods We retrospectively audited patients attending our day unit for epoprostenol infusions over a 12-month period between 2018 and 2019. Using our centre’s admissions database and electronic patient records, we identified which oral medications patients were currently co-prescribed or had previously trialled. Using pharmacy data and tariff costings, we calculated the cost of epoprostenol infusions and oral medications with blood monitoring. Results Between 2018 and 2019, 73 patients attended for epoprostenol infusions: 31 SSc, 25 RP, 17 other diagnoses (mixed/undifferentiated CTD, SLE, vasculitis). The mean number of epoprostenol infusions per patient per year was 5.92 days (range 1-25). The percentage of patients who had first been trialled on the following medications include: CCB 77.4%, ACEi/ARB 41.1%, fluoxetine 9.59%, sildenafil 87.1% and tadalafil 25.8%. In the SSc group 22.6% had also trialled bosentan. Only 2 SSc patients (6.45%) had trialled all of the drugs on the pathway prior to prostanoid reflecting the relative lack of efficacy of some first line therapies. The departmental tariff per prostanoid infusion is £450, resulting in an estimated average annual cost of £2700 per patient. The annual cost of supplying bosentan 125mg twice daily plus blood monitoring for the first year is approximately £1350. Conclusion Epoprostenol is used in our unit for patients with severe RP from a range of conditions. Sildenafil and CCB have been trialled in the majority of our patients prior to escalation. Only a minority of patients have received bosentan according to current guidelines and licensing. Given the reduction in cost, combined with the importance of avoiding hospital admissions with COVID-19, we would suggest that bosentan could be used earlier in the treatment pathway for a broader range of indications. NHSE is revising the SSc commissioning policy. Disclosure A. Young: None. B. Griffiths: None. J. Vila: None.

Objectives To assess the baseline care provided to patients with SLE attending UK Rheumatology units, audited against standards derived from the recently published BSR guideline for the management of adults with SLE, the NICE technology appraisal for belimumab, and NHS England’s clinical commissioning policy for rituximab. Methods SLE cases attending outpatient clinics during any 4-week period between February and June 2018 were retrospectively audited to assess care at the preceding visit. The effect of clinical environment (general vs dedicated CTD/vasculitis clinic and specialized vs non-specialized centre) were tested. Bonferroni’s correction was applied to the significance level. Results Fifty-one units participated. We audited 1021 episodes of care in 1003 patients (median age 48 years, 74% diagnosed >5 years ago). Despite this disease duration, 286 (28.5%) patients had active disease. Overall in 497 (49%) clinic visits, it was recorded that the patient was receiving prednisolone, including in 28.5% of visits where disease was assessed as inactive. Low documented compliance ( Conclusions This audit highlights significant unmet need for better disease control and reduction in corticosteroid toxicity and is an opportunity to improve compliance with national guidelines. Higher performance with nephritis screening in dedicated clinics supports wider adoption of this service-delivery model.

Rheumatology teams care for patients with diverse, systemic autoimmune diseases who are often immunosuppressed and at high risk of infections. The current COVID-19 pandemic has presented particular challenges in caring for and managing this patient group. The office of the chief medical officer (CMO) for England contacted the rheumatology community to provide expert advice on the identification of extremely vulnerable patients at very high risk during the COVID-19 pandemic who should be 'shielded'. This involves the patients being asked to strictly self-isolate for at least 12 weeks with additional funded support provided for them to remain at home. A group of rheumatologists (the authors) have devised a pragmatic guide to identifying the very highest risk group using a rapidly developed scoring system which went live simultaneous with the Government announcement on shielding and was cascaded to all rheumatologists working in England.

Background Scleromyxedema is a rare, idiopathic, generalised mucinosis with a progressive and unpredictable course, and has both cutaneous and systemic manifestations. It typically presents with a diffuse cutaneous papular eruption causing extensive skin tightening which can be severely debilitating and is one of four diagnostic criteria for scleromyxedema. The remaining criteria being evidence of histological mucinosis, presence of an IgG lambda monoclonal gammopathy and the absence of thyroid disease. Due to its rarity and subsequent lack of controlled studies, there remains no current standardised treatment. Relevant literature consists of case studies and series trialling use of a variety of immunosuppressive therapies with varying levels of success. Intravenous immunoglobulin (IVIG) has been shown to be the most effective therapeutic option so far and we report here further success following its use. Methods A previously healthy 54-year-old Caucasian woman presented to dermatology with widespread cutaneous mucinous deposits on her entire body with sparing of the anterior chest and abdomen. She was trialled on both topical and oral steroids as well as oral and subcutaneous methotrexate, but all had negligible benefit and her modified Rodnan’s skin score (mRss), an objective assessment of skin thickness, remained significantly raised at 29/51. A punch skin biopsy showed prominent superficial histiocytic mucinosis, and serum electrophoresis revealed an IgG lambda gammopathy, consistent with a diagnosis of scleromyxedema. To complete the diagnostic criteria, she also tested negative for thyroid dysfunction. In terms of systemic manifestations, she later presented with unexplained intermittent dysphagia and occasional reflux, which have previously been reported as common symptoms in 60-90% of patients with scleromyxoedema. Monthly treatment with IVIG at a dosage of 2 gram/kg over 2 days was started. Results The IVIG showed marked clinical success and minimal side effects. After four cycles, skin thickening was reversed and her mRss dropped to zero. Mycophenolate mofetil (MMF) (2g daily) was introduced after three months, to allow six-weekly IVIG infusions. Conclusion This report further supports the growing literature that IVIG is a safe and effective treatment for scleromyxedema. The underlying mechanism of how it treats scleromyxoedema is yet to be elucidated with only proposed mechanisms so far, such as the neutralisation of pathogenic autoantibodies. It is, however, important to acknowledge that IVIG is very expensive and in limited supply and requires time-consuming administration. To combat these downfalls, we had trialled the potential use of MMF as an IVIG ‘sparing agent’ to optimise patient convenience and reduce cost, without compromising clinical care. Unfortunately, the MMF was only successful for the first few months and was subsequently stopped due to adverse effects. Nevertheless, we still anticipate this work will provide further evidence for the approval of IVIG for the future treatment of scleromyxedema. Disclosures C. Lin None. F.A.H. Cooles None. S.J. Meggitt None. B. Griffiths None.

Background Tocilizumab was approved for the treatment of giant cell arteritis (GCA) by NICE in April 2018. This decision followed the GiACTA (Giant Cell Arteritis Actemra) study, a randomised control trial that demonstrated a beneficial effect of tocilizumab in reducing the frequency of disease flare and overall prednisolone requirements. However, as noted in the NICE appraisal, the extent to which the patient population in the GiACTA study reflects the population it is eventually used for in UK clinical practice is not clear. To address this, we analysed the records of all patients started on tocilizumab treatment for GCA in a single centre since its approval. Methods We performed a retrospective analysis of the clinical records of all patients started on tocilizumab for GCA at the Freeman Hospital in Newcastle. All patients are discussed in a regional connective tissue disease MDT prior to the initiation of therapy to ensure they fulfil NICE guidelines. At each subsequent visit adverse events related to both tocilizumab and corticosteroids and any flares of GCA or ischaemic events are recorded. Results In total 14 patients started tocilizumab since June 2018, with a cumulative exposure of 8 patient-years. The mean age was 74 years, slightly older than the average age in the GiACTA trial (69 years). In contrast to GiACTA where 48% of patients were newly diagnosed, all our patients had established disease. In our cohort 36% of patients had CT PET evidence of large vessel vasculitis, similar to the rate in GiACTA (40%) but higher than the national average of around 5%. Incidence of visual loss in our treated patients was higher at 29% than recorded in the general GCA population in the DC-VAS study (7.9%). In those treated for relapsing disease around half (56%) had recorded previous significant adverse events with steroids, including heart failure, hypertension and mood changes. Five patients had infections requiring antibiotics (cellulitis/ulcer in three, chest infection in one, urinary tract infections in two), with two serious infections requiring hospital admission (both urinary tract infections). No patients had further ischaemic events while on treatment. All patients were on a lower dose of prednisolone following treatment with an average of a 63% reduction in steroid dose. Conclusion The modest number of patients receiving tocilizumab for GCA suggests we are not treating everyone at first relapse. The higher rates of large vessel vasculitis, visual loss and previous steroid-induced complications suggest a preference for saving tocilizumab for a more severely affected subset of patients. In this preliminary data there were reassuringly no recorded ischaemic events following treatment and steroid doses were successfully reduced in all patients. Disclosures G. Reynolds None. B. Griffiths None. K. Houghton None. B. Thompson None. A.R. Lorenzi None. J. Heaney None.

Background The contemporary prevalence of ANCA-associated vasculitis (AAV) in England is unknown. Hospital Episode Statistics (HES) contain data on every hospital and day case NHS admission in England since 1997. In collaboration with the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS) we validated the diagnosis of AAV using ICD codes in HES. The positive predictive value of these codes was 86%, which compares favourably to a median coding accuracy of 80% in a recent systematic review of NHS coding studies. This justifies using this novel dataset for population-based epidemiology with coverage of the whole population of England. Methods We worked within NCARDRS enabled by their Section 251 legal permissions (CAG 10-02(d)/2015). We extracted all cases of AAV from HES 2011/12 to 2016/17 using ICD-10 codes M313 Granulomatosis with polyangiitis (GPA), M317 Microscopic polyangiitis (MPA), and M301 Eosinophilic granulomatosis with polyangiitis (EGPA). We used the Summary Care Record to check vital status and record date of death where appropriate. We estimated point prevalence on 1 July 2016 using ONS mid-year population estimates for England in 2016 as the denominator. Standardised mortality ratio (SMR) was calculated using the Office for National Statistics death summary tables 2016 to provide expected number of deaths for each 5-year age-band and sex. Results We identified 9,890 patients who were coded as having AAV during a hospital admission 2011-2017. This included 6,856 (69.3%) with GPA, 964 (9.8%) with MPA and 2,070 (20.9%) with EGPA. On 1 July 2016, our dataset found 8,040 people in England were living with ANCA associated vasculitis. We estimate the prevalence was 14.55 (95% CI: 14.23-14.87)/100,000 adult population. The median age of these patients was 65.3 years (interquartile range 52.3-74.2). 47% were female. The prevalence of GPA was 9.97/100.000 (95% CI: 9.71-10.24), MPA was 1.40/100,000 (95% CI: 1.30-1.50), and EGPA was 3.18/100,000 (95% CI: 3.03-3.33). People with AAV were 4.6 times more likely to die than the background population of the same age and sex (Standardised Mortality Ratio = 4.58). Conclusion There are no recent UK prevalence estimates for all types of ANCA-associated vasculitis. Studies in Australia, Germany, Southern Sweden and the USA have found estimated prevalence to be between 4.6-18.4 cases per 100,000 individuals. Our estimate of 14.6/100,000 in England is consistent with this, and towards the higher end of the range. However, our estimates underestimate the prevalence of MPA compared to other studies, and further work is needed to increase the routine identification of cases of MPA. Further work within NCARDRS using their unique data linkages will enable more specific AAV case ascertainment as well as nationwide population-based studies on cause of death and studies using the database of English prescriptions dispensed in the community. Disclosures F.A. Pearce None. B. Griffiths None. C. Mukhtyar None. R. Al-Jayoussi None. R.A. Watts None. J. Aston None. M. Bythell None. S. Stevens None. P.C. Lanyon None.

Background BD patients are managed equivalently by multiple medical specialties in our trust. Failure to switch from IV to s/c antiTNFalpha therapies was noted and our use of methotrexate, hydroxychloroquine and cyclophosphamide deviates from guidelines. Whether this effects outcome is unknown it but seems likely to be considered clinically useful. A national audit would determine if our management is an anomaly or in keeping with standard practice in the UK prompting review of our treatment algorithm. Methods Involved specialities provided details of BD patients in their care. Electronic case records from January 2009 to July 2019 were reviewed. Data were extracted using standard pro-forma classifying patients into 3 groups determined by their most severe manifestation: less severe (predominant musculoskeletal, mucocutaneous); ophthalmic (all eye disease) and severe disease (predominant CNS and vascular disease). Medication was assessed against guidelines for escalation and de-escalation protocols. Results 50 patients (representing approximately 7% of the national BD population) were identified from 4 specialties (Table 1). 23 were female; 45 identified as White British. 26 were regularly reviewed by 2 or more specialties, 4 had discontinued follow up. 26/50 had used antiTNFalpha therapies and 9/50 had received IV cyclophosphamide. Adherence to guidelines was determined by specialty and disease severity (Table 1). The EULAR guideline was followed least well by Rheumatology; low rates of adherence to the national guideline reflected frequent use of methotrexate and hydroxychloroquine in the less severe group and failure to switch to from IV to sub-cutaneous antiTNFalpha therapies. Deviations from national and EULAR guidelines in the ‘severe’ category related to the first line use of IV cyclophosphamide for acute organ/sight threatening disease. Conclusion BD patients are managed equivalently by multiple medical specialties in our Trust. Failure to switch from IV to s/c antiTNFalpha therapies was noted and our use of methotrexate, hydroxychloroquine and cyclophosphamide deviates from guidelines. Whether this effects outcome is unknown it but seems likely to be considered clinically useful. A national audit would determine if our management is an anomaly or in keeping with standard practice in the UK prompting review of our treatment algorithm. Disclosures J. Weightman None. B. Griffiths None. R. Pandit None. C. Stroud None. A.D. Price None. A.R. Lorenzi None.

Background The National Congenital Anomaly and Rare Disease Registration Service (NCARDRS) records those people with congenital anomalies and rare diseases across the whole of England. The NCARDRS Rare Rheumatology Project is establishing methodologies to identify and register people with rare rheumatic diseases. Hospital Episode Statistics (HES), comprising data from all admitted-patient NHS care in England, provides a promising source for case ascertainment. We aimed to confirm the reliability of diagnostic ICD codes for rare autoimmune rheumatic diseases within HES. Methods We used NCARDRS’ legal permissions (CAG 10-02(d)/2015) to extract cases who had at least one inpatient or daycase admission recorded in HES during 2003 to 2017 with a primary or additional diagnostic code for Kawasaki disease, Takayasu’s arteritis, Adult-onset Still’s disease, Relapsing polychondritis, Polyarteritis nodosa, Granulomatosis with polyangiitis (GPA), Microscopic polyangiitis (MPA) or Eosinophilic granulomatosis with polyangiitis (EGPA). Using Data Sharing Agreements between NCARDRS and NHS Trusts, we reviewed medical records for all cases admitted to one NHS Trust to confirm diagnoses. For ANCA-associated vasculitis (AAV), up to 20 randomly selected cases admitted to 5 Trusts with a diagnostic code of GPA, MPA and EGPA were reviewed. Results Table 1 shows the positive predictive value (PPV) of each diagnosis at one Trust: Kawasaki disease 100%, Takayasu’s arteritis 91.7%, Adult-onset Still’s disease 42.8%, Relapsing polychondritis 40.0%, and Polyarteritis nodosa 5.0%. For AAV, which had a PPV at the index Trust of 92.0%, the PPV across the 5 Trusts varied from 92.9% to 74.0%. Combining all 5 Trusts PPV for AAV was 85.9%. It was higher in MPA (PPV=98.4%) than in GPA (PPV=83.3%) and EGPA (PPV=77.4). Conclusion This is the first study to validate the coding of rare rheumatic disease diagnoses within HES data. Our results highlight heterogeneity in coding accuracy both between conditions and between NHS Trusts. Further work is needed to develop and validate algorithms for conditions where HES coding alone has poor positive predictive value, and test sensitivity. National rare disease registration will enable robust, cost-effective and reproducible population-based epidemiology of rare rheumatic diseases across England which is needed to inform service provision and aid development of new treatments. Disclosures F.A. Pearce None. M. Rutter None. B. Griffiths None. C. Mukhtyar None. R. Al-Jayoussi None. R.A. Watts None. J. Aston None. M. Bythell None. S. Stevens None. P.C. Lanyon None.

Background IgG4-related disease (IgG4-RD) is a multisystem immune mediated fibro-inflammatory condition characterised by the three histopathologic features of IgG4-RD lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. The condition can be indolent with few symptoms or present with organ or life-threatening disease. First-line treatment with high dose corticosteroids is often effective, however when tapering steroids, the disease can relapse, and second line agents such as methotrexate (MTX), azathioprine (AZA) or mycophenolate (MMF) are not always effective or tolerated. In 2016, following evidence from observational studies, NHS England approved the use of rituximab (RTX) in refractory IgG4-RD according to strict criteria. In our unit we have used rituximab, with or without cyclophosphamide induction, in eight patients with IgG4-RD. Our aim was to assess effectiveness of rituximab treatment and adherence to NHS England guidelines. Methods Using our connective tissue disease database, patients with IgG4-RD were identified and their electronic notes were reviewed. Outcome after rituximab treatment was assessed by the evaluation of clinical and radiological responses. Results Between August 2017 and September 2019, 15 patients with IgG4-RD were seen in the rheumatology service. 8 patients went on to receive rituximab therapy, 4 with IV cyclophosphamide (CYP). 5/8 patients had head and neck disease and 3/8 had abdominal disease (pancreas, retroperitoneal, renal). By comparison, those patients that did not receive rituximab had a preponderance of abdominal disease (4/7 had abdominal disease, 2/7 head and neck, 1/7 breast). 8/8 patients receiving rituximab were discussed in a designated MDT and met NHS diagnostic guidelines. MDT treatment decisions were made based on ‘refractory’ or ‘organ critical’ disease criteria. In all patients, with available post treatment imaging, radiological response was demonstrated. In one case, imaging showed improvement in some areas and progression in other areas and response was described as ‘partial’. Conclusion In the patients treated to date in our unit, rituximab has been shown to be an effective treatment for IgG4-RD. Cyclophosphamide induction has been used in a subset of patients. Patients with head and neck disease were more likely to require escalation to rituximab therapy, compared with those with abdominal disease. Disclosures F. Rayner None. B. Griffiths None. M. Nayar None. K. Oppong None. J. Vila None.

The British Society for Rheumatology guideline for the management of systemic lupus erythematosus in adults: Executive Summary.

Background:Low disease activity state has been defined using SLEDAI and used as treatment target in SLE. However, there has not been any such definition using BILAG-2004 index (BILAG-2004).Objectives:This study was to determine if low disease activity state according to BILAG-2004 is valid for use as treatment target in SLE. We also assessed disease activity longitudinally using BILAG-2004 systems tally (BST). BST is an alternative way of representing BILAG-2004 scores that combines the flexibility and simplification of numerical scoring of BILAG-2004 with the clinical intuitiveness of BILAG-2004 structure.Methods:This was a prospective multi-centre longitudinal study in the UK of an inception cohort of SLE patients (recruited within 12 months of achieving 1997 ACR revised criteria for SLE). Data were collected on disease activity (BILAG-2004 and BILAG2004-Pregnancy Index during pregnancy), SLICC/ACR DI (SDI), cumulative drug exposure and death at every visit. This study ran from 1st January 2005 to 31st December 2017. Four low disease activity states (LDA) were defined using BILAG-2004: 1) BILAG-2004 LDA when all 9 systems had scores of C, D or E on assessment (no Grade A or B), 2) BST LDA when there was persistent score of C, D or E in all 9 systems between 2 consecutive visits (equivalent to 2 consecutive visits with BILAG-2004 LDA), 3) BILAG-2004 Remission when all 9 systems had scores of D or E on assessment and 4) Persistent Remission when there was persistent score of D or E in all 9 systems between 2 consecutive visits. Longitudinal analysis using Poisson regression with random effects model was used with development of new damage as the outcome of interest. Gender, cardiovascular risk factors, antiphospholipid syndrome status and most drugs (except hydroxychloroquine, glucocorticoids, mycophenolate and cyclophosphamide) were excluded from the model as they were not associated with development of damage in univariate analysis.Results:273 patients were recruited (91.2% female, 59.3% Caucasian, 17.2% African/Caribbean, 17.2% South Asian) with mean age at recruitment of 38.5 years (SD 14.8). 97.8% had no damage at recruitment (2.2% had SDI score of 1). Median follow-up was 73.4 months (range: 1.8, 153.8) with total follow-up of 1767 patient-years. There were 13 deaths and 114 new damage items occurred during follow-up. There were 6674 assessments with disease activity score: 319 assessments with Grade A activity in 95 patients (84.6% had only 1 system with grade A, range: 1 - 4) and 1704 assessments with Grade A or B activity in 239 patients (78.7% had only 1 system with Grade A or B, range: 1 - 5).BILAG-2004 LDA was achieved in 74.5% of assessments (from 271 patients). BILAG-2004 Remission occurred in 28.2% of assessments (from 234 patients).6401 observations with BST were available (1 observation derived from change in activity between 2 consecutive assessments) and 63.7% were in BST LDA. There was no observation with Persistent Remission between consecutive visits.Table 1 summarises multivariate analysis which showed BILAG-2004 LDA to be inversely associated with damage. Similar results were obtained with BILAG-2004 Remission (RR 0.60 with 95% CI 0.38, 0.96) and BST LDA (RR 0.65 with 95% CI 0.43, 0.99). Cumulative drug exposure since recruitment for mycophenolate was protective against new damage (RR 0.99 with 95% CI 0.99, 0.99).Table 1.VariableRelative Risk (95% CI) for New DamageEthnicityAfro-Caribbean1.22 (0.68, 2.18)South Asian1.81 (0.97, 3.38)Others2.22 (0.63, 7.85)Age at diagnosis1.06 (1.04, 1.08)Prior SDI score0.68 (0.43, 1.06)BILAG-2004 LDA0.60 (0.39, 0.94)Hydroxychloroquine since last visit (per g)0.99 (0.98, 0.99)Steroids since last visit (per 100mg)1.02 (1.01, 1.03)Cyclophosphamide since last visit (per g)1.67 (1.15, 2.41)Conclusion:BILAG-2004 LDA and BST LDA are valid treatment targets in SLE. BILAG-2004 Remission and Persistent Remission are uncommon, which make them unrealistic as a treatment target.References:[1]Yee C. S., et al. The BILAG-2004 systems tally – a novel way of representing the BILAG-2004 index scores longitudinally. Rheumatology (Oxford) 2012; 51[11]: 2099-2105.Acknowledgements :Versus Arthritis, Vifor PharmaDisclosure of Interests:Chee-Seng Yee Consultant of: Bristol Myers Squibb, ImmuPharma, Grant/research support from: Vifor Pharma, Caroline Gordon Speakers bureau: UCB, Consultant of: Center for Disease Control, Astra-Zeneca, MGP, Sanofi and UCB, Mohammed Akil: None declared, Peter Lanyon: None declared, Christopher John Edwards Consultant of: Glaxo Smith Kline, Roche, Grant/research support from: Glaxo Smith Kline, Roche, David Isenberg: None declared, Anisur Rahman: None declared, Lee-Suan Teh: None declared, Sofia Tosounidou: None declared, Robert Stevens: None declared, Ahtiveer Prabu: None declared, Bridget Griffiths: None declared, Neil McHugh: None declared, Ian N. Bruce: None declared, Yasmeen Ahmad: None declared, Munther Khamashta: None declared, Vernon Farewell: None declared

Background:BILAG-2004 Index (BILAG-2004) has undergone construct and criterion validity and is used to assess disease activity in SLE. However, its predictive validity has yet to be established.Objectives:This study was to determine if disease activity according to BILAG-2004 was predictive of development of damage in an inception cohort.Methods:This was a prospective multi-centre longitudinal study in the UK of an inception cohort of SLE patients (recruited within 12 months of achieving 1997 ACR revised criteria for SLE). Data were collected on disease activity (BILAG-2004 and BILAG2004-Pregnancy Index during pregnancy), SLICC/ACR DI (SDI), cumulative drug exposure and death at every visit. Information on cardiovascular risk factors (hypertension, diabetes mellitus, hypercholesterolaemia and smoking status) and antiphospholipid syndrome status were also collected. This study ran from 1st January 2005 to 31st December 2017. Longitudinal analysis using Poisson regression with random effects model was used to determine predictors of development of new damage. Death was not included in the analysis due to small numbers.Results:273 patients were recruited (91.2% female, 59.3% Caucasian, 17.2% African/Caribbean, 17.2% South Asian) with mean age at recruitment of 38.5 years (SD 14.8). 97.8% had no damage at recruitment (2.2% had SDI score of 1). Median follow-up was 73.4 months (range: 1.8, 153.8) with total follow-up of 1767 patient-years. Prevalence of risk factors during follow-up were: hypertension 23.1%, hypercholesterolaemia 35.5%, diabetes mellitus 5.5%, smoker or ex-smoker 44% and antiphospholipid syndrome 7%. There were 13 deaths and 114 new damage items occurred during follow-up.There were 6674 assessments with disease activity score: 293 assessments with Grade A activity in 95 patients (92.4% had only 1 system with grade A, range: 1 - 4) and 1704 assessments with Grade A or B activity in 239 patients (78.7% had only 1 system with Grade A or B, range: 1 - 5).Univariate analysis showed that gender, cardiovascular risk factors, antiphospholipid syndrome and most drug exposure (except hydroxychloroquine, glucocorticoids, mycophenolate and cyclophosphamide) were not associated with new damage (they were not included in the multivariate analysis).Table 1 summarises multivariate analysis. Similar results were obtained when the disease activity variable was changed to Number of Systems with Grade A per assessment (RR 2.04 with 95% CI: 1.05, 3.94). Analysis using BILAG-2004 systems tally showed that persistent minimal disease was protective of development of damage (RR 0.74 with 95% CI: 0.57, 0.95). Cumulative drug exposure since recruitment for mycophenolate was protective against new damage (RR 0.99 with 95% CI 0.99, 0.99) but not cumulative drug exposure since last visit.VariableRisk Ratio (95% CI) for New DamageEthnicity Afro-Caribbean1.21 (0.68, 2.17) South Asian1.81 (0.97, 3.36) Others2.37 (0.68, 8.20)Age at diagnosis1.06 (1.04, 1.08)Prior SDI score0.69 (0.44, 1.08)Constitutional A or Bunreliable estimate due to low numbersMucocutaneous A or B1.80 (1.04, 3.14)Neuropsychiatric A or B4.68 (1.68, 13.05)Musculoskeletal A or B0.76 (0.33, 1.73)Cardiorespiratory A or B0.35 (0.05, 2.59)GIT A or Bunreliable estimate due to low numbersOphthalmic A or Bunreliable estimate due to low numbersRenal A or B2.08 (0.99, 4.40)Haematological A or B4.37 (1.15, 16.65)Hydroxychloroquine since last visit (per g)0.99 (0.98, 0.99)Prednisolone since last visit (per 100mg)1.01 (1.00, 1.02)Cyclophosphamide since last visit (per g)1.42 (0.94, 2.14)Conclusion:Active disease (Grade A or B) according to BILAG-2004 index is predictive of development of new damage in SLE patients.References:[1]Yee C. S., et al. The BILAG-2004 systems tally – a novel way of representing the BILAG-2004 index scores longitudinally. Rheumatology (Oxford) 2012; 51[11]: 2099-2105.Acknowledgements:Versus Arthritis and Vifor PharmaDisclosure of Interests:Chee-Seng Yee Consultant of: Bristol Myers Squibb, ImmuPharma, Grant/research support from: Vifor Pharma, Vernon Farewell: None declared, Mohammed Akil: None declared, Peter Lanyon: None declared, Christopher John Edwards Consultant of: Glaxo Smith Kline, Roche, Grant/research support from: Glaxo Smith Kline, Roche, David Isenberg: None declared, Anisur Rahman: None declared, Lee-Suan Teh: None declared, Sofia Tosounidou: None declared, Robert Stevens: None declared, Ahtiveer Prabu: None declared, Bridget Griffiths: None declared, Neil McHugh: None declared, Ian N. Bruce: None declared, Yasmeen Ahmad: None declared, Munther Khamashta: None declared, Caroline Gordon Speakers bureau: UCB, Consultant of: Center for Disease Control, Astra-Zeneca, MGP, Sanofi and UCB

The association of primary Sjögren’s syndrome (pSS) with Major Histocompatibility Complex (MHC) alleles is quintessential of MHC-disease associations. Indeed, although disease associations with classical HLA class I and II alleles/haplotypes are amply documented, further dissection is often prevented by the strong linkage disequilibrium across the entire MHC complex. Here we study the association of pSS, not with HLA genes, but with the non-conventional MHC encoded class I gene, MICA (MHC class I chain-related gene A). MICA is selectively expressed within epithelia, and is the major ligand for the activatory receptor, NKG2D, both attributes relevant to pSS’ etiology. MICA-pSS association was studied in two independent (French and UK) cohorts representing a total of 959 cases and 1,043 controls. MICA*008 allele was shown to be significantly associated with pSS (pcor=2.61 × 10−35). A multivariate logistic regression showed that this association was independent of all major known MHC-linked risk loci/alleles, as well as other relevant candidate loci that are in linkage disequilibrium with MICA*008 i.e. HLA-B*08:01, rs3131619 (T), MICB*008, TNF308A, HLA-DRB1*03:01 and HLA-DRB1*15:01 (P = 1.84 × 10−04). Furthermore, independently of the MICA*008 allele, higher levels of soluble MICA proteins were detected in sera of pSS patients compared to healthy controls. This study hence defines MICA as a new, MHC-linked, yet HLA-independent, pSS risk locus and opens a new front in our understanding of the still enigmatic pathophysiology of this disease. The fact that the soluble MICA protein is further amplified in MICA*008 carrying individuals, might also be relevant in other auto-immune diseases and cancer.

Introduction Enhanced recovery programmes are established as an essential part of laparoscopic colorectal surgery. Optimal pain management is central to the success of an enhanced recovery programme and is acknowledged to be an important patient reported outcome measure. A variety of analgesia strategies are employed in elective laparoscopic colorectal surgery ranging from patient-controlled analgesia to local anaesthetic wound infiltration catheters. However, there is little evidence regarding the optimal analgesia strategy in this cohort of patients. The LapCoGesic study aimed to explore differences in analgesia strategies employed for patients undergoing elective laparoscopic colorectal surgery and to assess whether this variation in practice has an impact on patient-reported and clinical outcomes. Materials and methods A prospective, multicentre, observational cohort study of consecutive patients undergoing elective laparoscopic colorectal resection was undertaken over a two-month period. The primary outcome measure was postoperative pain scores at 24 hours. Data analysis was conducted using SPSS version 22. Results A total of 103 patients undergoing elective laparoscopic colorectal surgery were included in the study. Thoracic epidural was used in 4 (3.9%) patients, spinal diamorphine in 56 (54.4%) patients and patient-controlled analgesia in 77 (74.8%) patients. The use of thoracic epidural and spinal diamorphine were associated with lower pain scores on day 1 postoperatively (P < 0.05). The use of patient-controlled analgesia was associated with significantly higher postoperative pain scores and pain severity. Discussion Postoperative pain is managed in a variable manner in patients undergoing elective colorectal surgery, which has an impact on patient reported outcomes of pain scores and pain severity.

ObjectivesTo report on fatigue in patients from the United Kingdom primary Sjögren’s syndrome (pSS) registry identifying factors associated with fatigue and robust to assignable causes such as comorbidities and medications associated with drowsiness.MethodsFrom our cohort (n = 608), we identified those with comorbidities associated with fatigue, and those taking medications associated with drowsiness. We constructed dummy variables, permitting the contribution of these potentially assignable causes of fatigue to be assessed. Using multiple regression analysis, we modelled the relationship between Profile of Fatigue and Discomfort physical and mental fatigue scores and potentially related variables.ResultsPain, depression and daytime sleepiness scores were closely associated with both physical and mental fatigue (all p ≤ 0.0001). In addition, dryness was strongly associated with physical fatigue (p ≤ 0.0001). These effects were observed even after adjustment for comorbidities associated with fatigue or medications associated with drowsiness.ConclusionsThese findings support further research and clinical interventions targeting pain, dryness, depression and sleep to improve fatigue in patients with pSS.This finding is robust to both the effect of other comorbidities associated with fatigue and medications associated with drowsiness.

Background The recently published UK guidelines for the management of SLE recommend biologic therapy for severe or refractory disease. The British Isles Lupus Assessment Group Biologics Register (BILAG-BR) has shown rituximab (RTX) to be safe, effective and corticosteroid [CS] sparing when used to treat refractory SLE. In 2013 NHS England published an interim clinical commissioning policy statement with criteria determining when RTX can be used to treat SLE. Objectives We evaluated our centre’s RTX retreatment strategy in patients with SLE and the consequent outcomes (disease activity and CS dose). Methods Records for the first 50 patients receiving RTX for refractory SLE who consented to join BILAG-BR from our centre between December 2013 and January 2016 were analysed (data cut off July 2016). Demographics, disease activityBILAG 2004/SLEDAI-2K), change in CS dose, retreatment schedules and adverse events were analysed. Results Median(IQR) age and disease duration were 42.8 (33–53) years and 9.5 (4–15.8) years respectively. Male: female ratio was 1:25. 80% were Caucasian, 6% Asian, 4% Caribbean and 10% other. All patients met SLICC/ACR classification criteria for SLE. The median(IQR) SLEDAI-2K scores and BILAG 2004 scores reduced from 6 (4–8) to 4 (0–4) (p 30/50 patients received their 1 st course of RTX at BILAG-BR baseline visit. 23 met criteria for active disease (at least 1A or 2Bs), 6 were taking an unacceptably high maintenance CS dose, and 1 was planning pregnancy. Median(IQR) CS dose in this group at baseline was 10 mg(1.5–20), and 5 mg(3–9) at 6 months. 70.8% demonstrated complete response at 6 months. 17 (57%) went on to have retreatment due to active disease, of which 11 (64.7%) had responded at 6 months post retreatment. Median(IQR) time to retreatment was 8 (6–12) months. 20/50 patients had received retreatment at predetermined intervals prior to their baseline BILAG-BR visit. Median(IQR) numbers of previous RTX courses were 3 (2–6). Median(IQR) CS dose was 8.75 mg(0–11.3) at baseline BILAG-BR visit and 5.5 mg(0–10) at 6 months. Median(IQR) time from baseline to retreatment was 6 (6–9.5) months. Median(IQR) sustained response was 18 (13.5–18) months. Conclusions Historically, our centre used time from 1 st treatment to flare as a guide to a patient’s future RTX retreatment schedule; patients were, on average, treated 2 months earlier than those treated under the current commissioning policy. Findings suggest that earlier retreatment led to sustained disease control and reduction of CS dose with no increase in adverse events. 11/16 incomplete responders responded following retreatment. Early retreatment may be associated with better outcomes for the patient and further research is needed in this area. Disclosure of Interest None declared