Elsevier

Autoimmunity Reviews

Volume 13, Issue 6, June 2014, Pages 621-629
Autoimmunity Reviews

Review
Patterns of systemic lupus erythematosus expression in Europe

https://doi.org/10.1016/j.autrev.2013.11.007Get rights and content

Abstract

Objectives

To analyse the differences in disease expression of European SLE patients based on gender, age at diagnosis, and ethnicity.

Methods

A two-year, retrospective, multicentre, observational study was carried out in five countries (France, Germany, Italy, Spain and the UK). Patients' clinical manifestations including disease activity, organ involvement, organ damage and flares were analysed.

Results

Thirty-one centres enrolled 412 consecutive eligible patients (90.5% of women), with active disease, stratified by disease severity (half severe and half non-severe).

Baseline characteristics included; mean (SD) age: 43.3 (13.6) years, SLE duration: 10.7 (8.0) years and age at disease diagnosis: 32.6 (13.0) years old. The mean (SD) SELENA-SLEDAI and SLICC/ACR scores were: 8.1 (6.7) and 0.82 (1.36), respectively. Over half of patients experienced flares (54.9%). The average number of annual flares was 1.01 (0.71) flares/year.

In males compared to females, the renal system was more frequently active (53.8% vs 30.0%, p = 0.002), the mean SLICC/ACR score was higher (1.15 vs 0.79, p = 0.039) and the pulmonary system was more likely to be damaged (12.8% vs 3.8%, p = 0.010). Furthermore, patients diagnosed at younger age displayed more renal system activity (young: 56.3% vs adult: 33.4% vs elder: 8.9%, p < 0.001) and renal damage (25.0% vs 6.9% vs 2.2%, p = 0.018) compared to the others. The annual number of flares (1.13 vs 1.05 vs 0.81 flares/year, p < 0.0001), including the occurrence of severe flares (0.58 vs 0.51 vs 0.20, p < 0.0001), was also higher in these patients.

Conversely, greater organ damage was observed in patients diagnosed at an older age compared to the others. The mean SLICC/ACR score was higher (1.31 vs young: 0.88 and adult: 0.78, p < 0.001) in patients diagnosed in the older age groups. The pulmonary (13.3% vs younger: 0% vs adult: 3.7%, p = 0.030) and cardiovascular (17.8% vs younger: 0% vs adult: 2.9%, p < 0.001) systems were more frequently damaged in these patients.

Black African descents showed greater disease activity compared to Caucasian patients. They flared more often (77.1% vs 48.6%, p = 0.001) and experienced a greater number of annual flares (1.57 vs 0.89 flares/year, p < 0.0001), mainly more severe flares (0.89 vs 0.38/year, p < 0.0001). They also were more likely to experience renal system damage.

Conclusion

The study showed clearly two patient subsets. The disease was the most active in Black African descents, and this phenomenon has never been described before in continental Europe. The disease was also more active in patients diagnosed at a younger or adult. Greater disease damage was observed in males and in patients diagnosed at an older age.

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

  • This LUCIE cohort, composed of SLE patients with active disease and half with severe disease, provides recent insights regarding SLE clinical profiles in Europe.

  • 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).

  • 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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