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Need for the guidelineDespite some improvement in survival data over the last 40 years (2;13), lupus patients still die on average 25 years earlier than the mean for women and men in the UK ( 16). The disease can present with slowly or rapidly progressive active disease at any age and can be associated with the rapid accumulation of damage if not promptly diagnosed, appropriately treated and regularly monitored (2;8;14;19;20). An up to date comprehensive guideline to optimise these aspects of management and consistent with current evidence and NHS practice, is warranted to improve the outcome of this variable and potentially life-threatening disease that causes considerable morbidity. There have been no previous UK based guidelines for lupus. The European (EULAR) recommendations for the management of lupus in general were not very detailed and were published in 2008( 22) although more specific recommendations were published for neuro-psychiatric lupus in 2010( 23) and joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ ERA-EDTA) recommendations for lupus nephritis were published in 2012( 24), as well as American College of Rheumatology (ACR) guidelines for the management of lupus nephritis in 2012(25). Objectives of the guidelineThe aim of this guideline was to produce recommendations for the management of adult lupus patients in the UK that cover the diagnosis, assessment, and monitoring of lupus and the treatment of mild, moderate and severe active lupus disease but that do not imply a legal obligation. The resulting recommendations are based on an extensive literature review up to June 2015 to produce evidence-based guidelines, particularly for the treatment of non-renal lupus, supplemented as necessary by expert opinion and consensus agreement (tables 1 and 2). The guideline development group recommend that patients with lupus nephritis are managed according to the EULAR/ERA-EDTA recommendations for lupus nephritis(24) and provide their levels of agreement with a summary of the most important items in those recommendations (table 3). Target population, target audience and stakeholder involvementThe guidelines address the management of adult patients only and have been developed by a multidisciplinary guideline development group set up by the BSR and led by CG, consisting of academic (CG, INB, DDC, MK, DI) and NHS consultants in rheumatology (MA, BG) and nephrology (DJ, LL), rheumatology trainees (MG, KS), a GP (BE) and a clinical nurse specialist (SB), a patient representative (YN) and a lay member (PN). All participants declared any conflicts of interest and these are listed at the end of this article. The target audience includes rheumatologists and other clinicians such as nephrologists, immunologists and dermatologists, trainees in these specialties and emergency medicine, GPs, clinical nurse specialists, and other allied health professionals involved in the care of adult lupus patients. Opinions of BSR SLE Full guideline final with...

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Individuals with systemic lupus erythematosus (SLE) have an increased susceptibility to certain types of cancer. Of particular concern are haematologic malignancies, specifically non-Hodgkin lymphoma, where a three- to four-fold increased risk is seen in SLE, compared with the general population. There is some evidence that immunosuppressive exposures play a role, although there appear to be other factors driving the risk. Lupus disease activity, with resultant dysregulated lymphocyte proliferation, may itself be a mediator of the association between SLE and lymphoma. Aside from haematologic malignancy risk, lung cancer also is increased in SLE compared with the general population, and smoking likely drives this risk in large part. Last but not least, cervical dysplasia is a concern in women with SLE, particularly with exposure to immunosuppressants; routine screening for this complication should not be neglected.

NICE has accredited the process used by BSR to create its clinical guidelines. The term began on 27 February 2012 and the current renewed accreditation is valid until 31 December 2023. More information on accreditation can be viewed at www.nice.org.uk/accreditation.

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Improvement of the overall stability of SLE and early intervention to minimize the impact of renal disease may be two approaches to mitigate the long-term direct cost of managing SLE patients in the UK.

Background: Autoantibodies can be identified in approximately 60% of UK children with JDM. With the development of quantitative techniques such as ELISA to detect these autoantibodies there is a growing interest in the clinical utility of autoantibodies in predicting disease activity and the risk of associated complications. To date, the titre of anti-MDA5 has been shown to be useful in predicting response to treatment in Japanese children with JDM and in adults small studies have also shown a relationship between the titres of anti-Jo-1, anti-MDA5, anti-HMGCR and anti-SRP with disease activity measures. Methods: Serum samples and matched clinical data were obtained from 285 patients with JDM recruited to the UK Juvenile Dermatomyositis Cohort and Biomarker Study. After initial screening for the presence of autoantibodies by immunoprecipitation, the antibody titre of those with anti-TIF1g, anti-NXP2 and anti-MDA5 was assessed by ELISA using recombinant antigen. First and last available samples were analysed for each autoantibody subgroup (32 patients with anti-TIF1g, 30 with anti-NXP2 and 15 with anti-MDA5) along with multiple serial samples in those where an initial sample was available within 6 months of diagnosis (12 patients with anti-TIF1g, 8 with anti-NXP2 and with 4 anti-MDA5). Between three and eight samples were available per patient. Results: Autoantibody titre was seen to change over the follow-up period and reduced in the majority of patients in all autoantibody subgroups. In the last available sample (taken 1-19 years post diagnosis) autoantibody titre had reduced below the ELISA positive threshold in eight (25%) patients with anti-TIF1g, seven (23%) with anti-NXP2 and eight (53%) with anti-MDA5. Analysis of serial samples showed a relationship between autoantibody titre and change in disease activity as measured by the physician global assessment score (PGAS). While numbers were too small to demonstrate statistical significance within individual patients, trends were striking and a strong positive correlation was confirmed. Conclusion: Autoantibody titre in JDM changes over time and reduces in the majority of patients. Within individual patients autoantibody titre correlates with disease activity as measured by the Physician Global Assessment Score. A corresponding rise in autoantibody titre to mirror Physician Global Assessment Score in those patients who relapsed, suggests that this does not simply represent regression to the mean. Limitations of this study include few patients with serial samples available, variably timed blood sampling and non-standardized treatment. Further work is needed to confirm these results and to assess whether alterations in autoantibody titre pre-date changes in disease activity; if so, and given the clinical sub-phenotypes associated with these antibodies, the routine testing of autoantibodies early in diagnostic workup is to be recommended. Disclosure statement: S.L.T. has received research support in the form of a BMA Doris Hillier Grant 2012. All other authors have...