isrctn.org Identifier: ISRCTN54371254.

Some significant changes in the BILAG disease activity index to assess patients with SLE are proposed. The process of demonstrating validity and reliability has started with these two exercises assessing real patients. Further validation studies are under way. BILAG 2004 is likely to be valuable in clinical trials assessing new therapies for the treatment of SLE, as it provides a more comprehensive system-based disease activity measure than has been available previously.

Objective. To develop and validate a disease-specific health-related quality of life (HRQOL) instrument for adults with systemic lupus erythematosus (SLE).Methods. The work consisted of 6 stages. Stage 1 included item generation for questionnaire content from semistructured interviews with SLE patients. In stage 2 item selection for the draft questionnaire was performed by thematic analysis of the patient interview transcripts and expert panel agreement. In stage 3 the content validity of the draft questionnaire was assessed by patients completing the questionnaire and providing critical feedback. In stages 4 and 5 construct validity and internal reliability of the 3 versions of the LupusQoL were evaluated using principal component analysis with varimax rotation and Cronbach's alpha coefficients, respectively. In stage 6 discriminatory validity, concurrent validity, and test-retest reliability were evaluated.

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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