ReviewPatterns of systemic lupus erythematosus expression in Europe
Introduction
Systemic lupus erythematosus (SLE) is an autoimmune disease characterised by alternating periods of remission and disease activity, mainly expressed by flare occurrence. Patients may present with a wide variety of clinical manifestations and immunological features due to the numerous organs that may be involved with activity or damage (skin, joints, kidneys, lungs, heart and brain) [1], [2]. SLE is treated with a combination of treatments including corticosteroids, non-steroidal anti-inflammatory drugs (NSAIDs), antimalarials, immunosuppressants [3], [4], and biologicals (used in patients with refractory disease in Europe) [5].
Several European and international studies, which analysed patients' long term outcome, showed that some factors (e.g. younger age at disease onset [6], [7], [8], male gender [6], [7], autoantibody patterns [6], [7], [9], treatment class [8], [10] or renal involvement [11]) could predispose patients to a less favourable disease outcome and be prognostic of morbidity and mortality. The Euro-Lupus study was conducted in 1000 SLE European patients followed during a 10-year period. The survival probability at 10 years of patients presenting with nephropathy at inclusion in the study was lower compared to that of patients without nephropathy (88% vs 94%, p = 0.045) [11]. Furthermore, the multiethnic cohorts GLADEL (Grupo Latinoamericano de Estudio del Lupus) and LUMINA (Lupus in Minority Populations, Nature versus Nurture), conducted in 1214 and 635 patients respectively, identified disease activity and damage as the most important determinants of mortality [8], [10]. Moreover, younger age at disease onset [10], [12], renal and cardiovascular involvement, African or Latin American mestizo heritage, lower use of antimalarials and higher use of immunosuppressants were also described as predictors of poor outcome [8].
Even if the overall prognosis of SLE has improved in the last fifteen years due to better disease management [13], [14], [15], the long term prognosis still remains less favourable for some patients due to the clinical complications of the disease and/or its treatments [15]. Thus patient's clinical feature assessment could be helpful for SLE diagnosis, anticipate long term prognosis and use the most accurate treatments in order to improve SLE care management. These clinical features may be associated with particular disease characteristics, influence disease outcome and define specific clinically significant patients' subsets.
The aim of this manuscript was to analyse the main clinical manifestations based on patients demographic and clinical characteristics, including: gender, age at diagnosis, and ethnicity, in order to characterise the various patterns of SLE expression. The study results are compared to the large international lupus cohorts, specifically the European cohort Euro-Lupus.
Section snippets
Study design
The LUCIE (LUpus erythematosus Cost of Illness in Europe) study was initially designed to describe the profile of adult SLE patients with active, autoantibody positive disease, and to evaluate the annual direct medical cost associated with SLE. LUCIE is a two-year multi-centre, retrospective study (October 2010–April 2011) based on data reported in patients' medical records. The study was carried out in five European countries (France, Germany, Italy, Spain and the UK), and included 26
Results
The study sample included 412 eligible patients (25 patients were excluded because of noncompliance with eligibility criteria). The mean follow-up study duration was 24.7 (3.2) months.
Discussion
The LUCIE study examined differences in SLE patients' main clinical manifestations (organ involvement, organ damage, disease activity and flare characteristics) in five European countries. Two types of patients' subsets were clearly identified: increased disease activity was observed in Black African patients and those with younger or adult age at disease diagnosis, whereas greater damage was reported in males and in patients with elder age at disease diagnosis.
The LUCIE cohort included SLE
Conclusion
The LUCIE cohort, composed of patients with active disease and half with severe disease, provides recent insights regarding SLE clinical profiles in Europe. The findings are in line with the current international overall knowledge [34], [35], [36], [37], [38], [39], [40]. Two distinct patients' clinical subsets were identified; one with higher disease activity (Black African patients and young disease diagnosis) and the other with higher disease damage (males and elder disease diagnosis), as
Funding
The LUCIE study was funded by the GlaxoSmithKline (Uxbridge, UK) and Human Genome Sciences, Inc. (Rockville, Maryland, USA) grant number: study GHO-09-2521, etrack number (BEL114431).
Contributors
All authors participated actively in conducting the study. They contributed to critical revisions to the content of the manuscript and have all approved the article for publication.
Competing interests
The Scientific Committee, composed of clinical (RC, AD, ZA, MK, MS and JR), epidemiological (FG) and medico-economic (GK) experts, has received payment for board membership and consultancy from GlaxoSmithKline and Human Genome Sciences.
RC has received payment for lectures and educational presentations from GlaxoSmithKline and for board membership from Roche, Medimmune, UCB, Novo Nordisk and Lilly. AD has received payment for lectures from GlaxoSmithKline and UCB. ZA was invited to ACR 2011 and
Take-home messages
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This LUCIE cohort, composed of SLE patients with active disease and half with severe disease, provides recent insights regarding SLE clinical profiles in Europe.
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Two distinct patients' clinical subsets were identified: one with higher disease activity (Black African patients and young disease diagnosis) and the other with higher disease damage (males and elder disease diagnosis).
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Strategies including treatments tailored to the patient's clinical profile should be applied as soon as possible since
Acknowledgements
We thank all the investigators for their active participation in the study ensuring adequate patient recruitment.
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