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Original research
Systemic lupus erythematosus and neutropaenia: a hallmark of haematological manifestations
  1. Aurore Meyer1,2,
  2. Aurélien Guffroy1,2,3,
  3. Gilles Blaison4,
  4. Yannick Dieudonne1,2,3,
  5. Zahir Amoura5,
  6. Bernard Bonnotte6,
  7. Christoph Fiehn7,
  8. Pierre Kieffer8,
  9. Hannes Martin Lorenz9,
  10. Nadine Magy-Bertrand10,
  11. François Maurier11,
  12. Jean-Louis Pennaforte12,
  13. Hans-Hartmut Peter13,
  14. Andreas Schwarting14,
  15. Jean Sibilia15,
  16. Laurent Arnaud2,3,15,
  17. Thierry Martin1,2,3,
  18. Reinhard Edmund Voll13 and
  19. Anne-Sophie Korganow1,2,3
  20. and the LBBR/Rarenet group
    1. 1Department of Clinical Immunology and Internal Medicine, National Reference Center for autoimmune diseases (RESO), Strasbourg University Hospital, Strasbourg, France
    2. 2UFR Médecine, Université de Strasbourg, Strasbourg, France
    3. 3INSERM UMR - S1109, Faculté de Médecine, Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France
    4. 4Department of Internal Medicine, Hôpitaux Civils de Colmar, Colmar, Alsace, France
    5. 5Department of Internal Medicine, Institut E3M, Assistance Publique Hôpitaux de Paris (APHP), Groupement Hospitalier Pitié Salpêtrière, Paris, France
    6. 6Department of Clinical Immunology and Internal Medicine, CHU Dijon Bourgogne, Dijon, France
    7. 7ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany
    8. 8Department of Internal Medicine, Centre hospitalier de Mulhouse, Mulhouse, France
    9. 9Department of Medicine V, University Hospital Heidelberg, Center for Rheumatic Diseases Baden-Baden, Heidelberg, Germany
    10. 10Department of Internal Medicine, CHU de Besançon, Besançon, France
    11. 11Department of Internal Medicine, Hôpitaux Privés de Metz, Metz, France
    12. 12Department of Internal Medicine, CHU de Reims, Hôpital Robert Debré, Reims, France
    13. 13Department of Rheumatology and Clinical Immunology, Medical Center - Faculty of Medicine, University of Freiburg, Freiburg, Germany
    14. 14Department of Internal Medicine, Universitätsmedizin, Mainz, Germany
    15. 15Department of Rheumatology, National Reference Center for autoimmune diseases (RESO), Hôpitaux universitaires de Strasbourg, Strasbourg, France
    1. Correspondence to Dr Aurélien Guffroy; aurelien.guffroy{at}


    Objective Systemic lupus is a chronic autoimmune disease characterised by its phenotypic heterogeneity. Neutropaenia is a frequent event in SLE occurring in 20%–40% of patients depending on the threshold value of neutrophil count. On a daily basis, the management of neutropaenia in SLE is difficult with several possible causes. Moreover, the infectious consequences of neutropaenia in SLE remain not well defined.

    Methods 998 patients from the Lupus BioBank of the upper Rhein (LBBR), a large German and French cohort of patients with SLE, mostly of Caucasian origin (83%), were included in this study. Neutropaenia was considered when neutrophil count was below 1800×106/L. An additional analysis of detailed medical records was done for 65 LBBR patients with neutropaenia.

    Results 208 patients with neutropaenia (21%) were compared with 779 SLE patients without neutropaenia. Neutropaenia in SLE was significantly associated with thrombocytopaenia (OR 4.11 (2.57–10.3)), lymphopaenia (OR 4.41 (2.51–11.5)) and low C3 (OR 1.91 (1.03–4.37)) in multivariate analysis. 65 representative patients with neutropaenia were analysed. Neutropaenia was moderate to severe in 38%, chronic in 31%, and both severe and chronic in 23% of cases. Moderate to severe and chronic neutropaenia were both associated with lymphopaenia and thrombopaenia. Chronic neutropaenia was also associated anti-Ro/SSA antibodies and moderate to severe neutropaenia with oral ulcers.

    Conclusion This study is to date the largest cohort to describe neutropaenia in SLE. Neutropaenia displays a strong association with other cytopaenias, suggesting a common mechanism. Chronic neutropaenia is associated with anti-Ro/SSA antibodies with or without identified Sjögren’s disease.

    • lupus erythematosus, systemic
    • autoimmune diseases
    • autoantibodies

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    • Collaborators LBBR/Rarenet group: Z Amoura (APHP, Paris), L Arnaud (Strasbourg), G Blaison (Colmar), B Bonnotte (Dijon), E Chatelus (Strasbourg), E Ciobanu (Mulhouse), F Duchene (Belfort), JP Faller (Belfort), A Gorse (Strasbourg), JE Gottenberg (Strasbourg), O Hinschberger (Mulhouse), F Jaeger (Mulhouse), P Kieffer (Mulhouse), M Kilifa (Strasbourg), N Magy-Bertrand (Besançon), T Martin (Strasbourg), L Martzolff (Mulhouse), F Maurier (Metz), A Meyer (Strasbourg), J-L Pasquali (Strasbourg), J-L Pennaforte (Reims), V Poindron (Strasbourg), S Revuz (Metz), M Samson (Dijon), J Sibilia (Strasbourg), C Sordet (Strasbourg), A Theulin (Strasbourg), D Wahl (Nancy), JC Weber (Strasbourg), M Bartsch (Freiburg), N Bartholomä (Freiburg), C Fiehn (Baden-Baden), S Finzel (Freiburg), A Funkert (Heidelberg), M Hausberg (Karlsruhe), H Lorenz, R Max (Heidelberg), H-H Peter (Freiburg), M Rizzi (Freiburg), A Schwarting (Mainz), J Thiel (Freiburg), N Venhoff (Freiburg), R Voll (Freiburg).

    • Contributors A-SK, REV and AG designed the study. AM, AG, GB, YD, ZA, BB, CF, PK, HML, NM-B, FM, J-LP, H-HP, AS, JS, LA, TM, REV and A-SK collected the data and performed the study analyses. AM, AG, YD and A-SK wrote the manuscript. All authors read and approved the final version of the manuscript.

    • Funding This study was supported by grants from EU-funded (ERDF) project INTERREG IV and V 'LBBR' and 'RARENET'.

    • Competing interests None declared.

    • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

    • Patient consent for publication Not required.

    • Ethics approval The study complies with the Declaration of Helsinki and was approved by the appropriate medical ethical committees.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Data availability statement All data relevant to the study are included in the article or uploaded as supplementary information.

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