Over the last 4 years, since the last issue of Best Practice and Research in Clinical Rheumatology devoted to systemic lupus erythematosus (SLE), there have been significant advances in the assessment, management and prognosis of this multisystem auto-immune rheumatic disease.

We have invited contributors who are recognised experts in their fields to provide updates on aspects of lupus that clinicians often find challenging in clinical practice. For each of the topics, specific questions have been posed to the authors to focus the review and provide a clinically relevant update for the busy clinician.

For some topics such as lupus nephritis, there have been a series of major randomised controlled trials that have changed the management of this life-threatening complication. Significantly, there has been a move away from high-dose cyclophosphamide-containing induction regimens towards low-dose cyclophosphamide approaches. More recently, cyclophosphamide-free induction regimens are now being widely used, with mycophenolate mofetil for both induction and maintenance becoming the standard of care. Similarly, there is a recognition that corticosteroid doses are perhaps too high and result in unacceptable adverse effects including damage accumulation. With pioneering steroid-free approaches, there may come a time when steroids are used in minimal doses when combined with biologic agents, or even avoided altogether. However, therapies only work if patients take the drugs prescribed to them and the reasons for non-adherence are varied and many. Non-adherence to therapy is probably the single most common cause of lupus flares, admission to hospital and progression to end-stage renal failure, and this important topic has therefore been addressed in this edition.

By contrast, the assessment and management of patients with central nervous system disease is striking by the complete absence of controlled trials – indeed, these patients are specifically excluded from industry-led clinical trials. Many clinicians find these patients especially challenging and patients with demyelinating disease in the context of lupus are particularly difficult to assess. Do these patients have coincident multiple sclerosis or is the demyelination an intrinsic complication of SLE? There are no easy answers but this question has specifically been addressed.

Clinical trials of new therapies in SLE have been a major disappointment over the last decade with many negative randomised controlled trials. There have however, been notable successes with belimumab and potentially exciting new biologic therapies such as epratuzumab and others in early phase clinical trials. One of the problems with the early clinical trials is that the tools used to assess disease activity, such as British Isles Lupus Activity Group (BILAG) and SLE Disease Activity Index (SLEDAI), were not designed for use in large-scale trials. Capturing disease response, defining lupus flares and measuring damage accumulation have therefore become critically important and these aspects have been reviewed.

Pregnancy and the assessment of neonatal lupus and paediatric-onset lupus are of central importance to patients, obstetricians, neonatologists, paediatricians and rheumatologists and these topics have been updated.

When patients are asked about their most important and troublesome symptom, rheumatologists often expect them to answer in terms of problems such as chronic pain, arthritis or skin disease. However, fatigue and impaired quality of life are by far the most commonly reported problems that have a day-to-day impact on patients. Attempts have been made to try to measure and understand the factors responsible and address treatment strategies for these most challenging disease-related quality of life issues.

Infection in patients with lupus not only leads to significant morbidity including hospital admission but also can, in some circumstances, be fatal. The advice on immunisation of patients with SLE has changed substantially from avoidance of immunisation for fear of triggering a lupus flare. Most rheumatologists now advocate widespread immunisation of patients, especially those at increased risk of infection, such as those on immunosuppressive therapies or who have immunoglobulin deficiencies as a result of long-term immunosuppression. The advent of B-cell-depleting therapies is likely to result in an increase in such infectious complications.

Finally, although pulmonary hypertension is a rare complication of SLE, in general the prognosis is better than in patients with systemic sclerosis. Increased awareness of pulmonary hypertension in SLE is leading to earlier diagnosis with right-heart catheterisation and the use of advanced therapies has improved the outcome for these patients.

This volume of Best Practice and Research in Clinical Rheumatology devoted to SLE is aimed at junior and senior clinicians who may not be lupus specialists, but we hope that even colleagues who are interested in this fascinating disease may find the updates of interest.