The British Society for Rheumatology guidelines for the management of systemic lupus erythematosus in adults

Research output: Contribution to journalArticlepeer-review

Authors

  • Maame-Boatemaa Amissah-Arthur
  • Mary Gayed
  • Sue Brown
  • Ian N Bruce
  • David D'Cruz
  • Ben Empson
  • Bridget Griffiths
  • David Jayne
  • MA Khamashta
  • Liz Lightstone
  • Peter Norton
  • Yvonne Norton
  • Karen Schreiber
  • David Isenberg

Colleges, School and Institutes

External organisations

  • Central Manchester University Hospital NHS Foundation Trust and Manchester Academic Health Science Centre
  • University of Manchester
  • Christie Hospital NHS Foundation Trust
  • Community Rheumatology department
  • Newcastle upon Tyne Hospitals NHS Foundation Trust
  • University of Cambridge
  • Rayne Institute and St Thomas' Hospital London, UK.
  • University College London Hospitals NHS Foundation Trust

Abstract

SLE (or lupus for short) is a multisystem, autoimmune disease, involving complex pathogenetic mechanisms that can present at any age. It most commonly presents in women in the reproductive age group, although lupus is increasingly recognized after the age of 40 years, particularly in Europeans. Lupus affected nearly 1 in 1000 of the population in the UK in 2012 and was most frequently observed in people of AfricanCaribbean and South Asian descent [46]. The age-standardized incidence in the UK according to the Clinical
Practice Research Datalink is 8.3/100 000/year for females and 1.4/100 000/year for males, and the highest incidence rates are seen in those of African Caribbean descent: 31.4/100 000/year, compared with 6.7/100 000/year for those of white European descent.

The mean age at diagnosis is 48.9 years, but it is lower in those of African ancestry in the UK and North America. The disease is prone to relapses and remissions, resulting in considerable morbidity due to flares of disease activity and accumulated damage, and an increased risk of premature death, mostly due to infection or cardiovascular disease. Death from active lupus is rare in the
UK; however, a 10% mortality over 20 years and a mean age of death of 53.7 years was recently reported. About one-third of SLE patients in the UK develop
LN. Patients of African ancestry tend to present young with LN in the UK, as in the USA and elsewhere, and are at considerable risk of developing endstage renal disease (ESRD) and of dying prematurely. In another UK cohort, ESRD occurred in 20% of LN patients within 10 years of diagnosis, and the mean age at death in LN patients was 40.3 years, with an average of 7.5 years between development of LN and death.

The mainstay of therapy for active lupus until recently has been NSAIDs, CSs, antimalarials such as HCQ, and immunosuppressants such as AZA and CYC, although only prednisolone and HCQ are licensed for lupus. With the exception of LN, there were relatively few trials until the last 15 years, and in 2011, belimumab became the first drug to be licensed for the treatment of active lupus for over 50 years. New therapies that will reduce the need for CSs to control lupus activity and to reduce the development of damage and infection are
needed to improve outcome. In the meantime it is important to manage patients optimally with the treatment strategies that are available.

Details

Original languageEnglish
Pages (from-to)E1-E45
Number of pages45
JournalRheumatology (Oxford, England)
Volume57
Issue number1
Publication statusPublished - 6 Oct 2017

Sustainable Development Goals