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Systemic lupus erythematosus and neutropaenia: a hallmark of haematological manifestations.
Lupus Sci Med. 2020 07; 7(1)LS

Abstract

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.

Authors+Show Affiliations

Department of Clinical Immunology and Internal Medicine, National Reference Center for autoimmune diseases (RESO), Strasbourg University Hospital, Strasbourg, France. UFR Médecine, Université de Strasbourg, Strasbourg, France.Department of Clinical Immunology and Internal Medicine, National Reference Center for autoimmune diseases (RESO), Strasbourg University Hospital, Strasbourg, France aurelien.guffroy@chru-strasbourg.fr. UFR Médecine, Université de Strasbourg, Strasbourg, France. INSERM UMR - S1109, Faculté de Médecine, Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France.Department of Internal Medicine, Hôpitaux Civils de Colmar, Colmar, Alsace, France.Department of Clinical Immunology and Internal Medicine, National Reference Center for autoimmune diseases (RESO), Strasbourg University Hospital, Strasbourg, France. UFR Médecine, Université de Strasbourg, Strasbourg, France. INSERM UMR - S1109, Faculté de Médecine, Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France.Department of Internal Medicine, Institut E3M, Assistance Publique Hôpitaux de Paris (APHP), Groupement Hospitalier Pitié Salpêtrière, Paris, France.Department of Clinical Immunology and Internal Medicine, CHU Dijon Bourgogne, Dijon, France.ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany.Department of Internal Medicine, Centre hospitalier de Mulhouse, Mulhouse, France.Department of Medicine V, University Hospital Heidelberg, Center for Rheumatic Diseases Baden-Baden, Heidelberg, Germany.Department of Internal Medicine, CHU de Besançon, Besançon, France.Department of Internal Medicine, Hôpitaux Privés de Metz, Metz, France.Department of Internal Medicine, CHU de Reims, Hôpital Robert Debré, Reims, France.Department of Rheumatology and Clinical Immunology, Medical Center - Faculty of Medicine, University of Freiburg, Freiburg, Germany.Department of Internal Medicine, Universitätsmedizin, Mainz, Germany.Department of Rheumatology, National Reference Center for autoimmune diseases (RESO), Hôpitaux universitaires de Strasbourg, Strasbourg, France.UFR Médecine, Université de Strasbourg, Strasbourg, France. INSERM UMR - S1109, Faculté de Médecine, Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France. Department of Rheumatology, National Reference Center for autoimmune diseases (RESO), Hôpitaux universitaires de Strasbourg, Strasbourg, France.Department of Clinical Immunology and Internal Medicine, National Reference Center for autoimmune diseases (RESO), Strasbourg University Hospital, Strasbourg, France. UFR Médecine, Université de Strasbourg, Strasbourg, France. INSERM UMR - S1109, Faculté de Médecine, Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France.Department of Rheumatology and Clinical Immunology, Medical Center - Faculty of Medicine, University of Freiburg, Freiburg, Germany.Department of Clinical Immunology and Internal Medicine, National Reference Center for autoimmune diseases (RESO), Strasbourg University Hospital, Strasbourg, France. UFR Médecine, Université de Strasbourg, Strasbourg, France. INSERM UMR - S1109, Faculté de Médecine, Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France.No affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

32616563

Citation

Meyer, Aurore, et al. "Systemic Lupus Erythematosus and Neutropaenia: a Hallmark of Haematological Manifestations." Lupus Science & Medicine, vol. 7, no. 1, 2020.
Meyer A, Guffroy A, Blaison G, et al. Systemic lupus erythematosus and neutropaenia: a hallmark of haematological manifestations. Lupus Sci Med. 2020;7(1).
Meyer, A., Guffroy, A., Blaison, G., Dieudonne, Y., Amoura, Z., Bonnotte, B., Fiehn, C., Kieffer, P., Lorenz, H. M., Magy-Bertrand, N., Maurier, F., Pennaforte, J. L., Peter, H. H., Schwarting, A., Sibilia, J., Arnaud, L., Martin, T., Voll, R. E., & Korganow, A. S. (2020). Systemic lupus erythematosus and neutropaenia: a hallmark of haematological manifestations. Lupus Science & Medicine, 7(1). https://doi.org/10.1136/lupus-2020-000399
Meyer A, et al. Systemic Lupus Erythematosus and Neutropaenia: a Hallmark of Haematological Manifestations. Lupus Sci Med. 2020;7(1) PubMed PMID: 32616563.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Systemic lupus erythematosus and neutropaenia: a hallmark of haematological manifestations. AU - Meyer,Aurore, AU - Guffroy,Aurélien, AU - Blaison,Gilles, AU - Dieudonne,Yannick, AU - Amoura,Zahir, AU - Bonnotte,Bernard, AU - Fiehn,Christoph, AU - Kieffer,Pierre, AU - Lorenz,Hannes Martin, AU - Magy-Bertrand,Nadine, AU - Maurier,François, AU - Pennaforte,Jean-Louis, AU - Peter,Hans-Hartmut, AU - Schwarting,Andreas, AU - Sibilia,Jean, AU - Arnaud,Laurent, AU - Martin,Thierry, AU - Voll,Reinhard Edmund, AU - Korganow,Anne-Sophie, AU - ,, PY - 2020/03/23/received PY - 2020/06/03/revised PY - 2020/06/04/accepted PY - 2020/7/4/entrez PY - 2020/7/4/pubmed PY - 2020/7/4/medline KW - autoantibodies KW - autoimmune diseases KW - lupus erythematosus, systemic JF - Lupus science & medicine JO - Lupus Sci Med VL - 7 IS - 1 N2 - 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. SN - 2053-8790 UR - https://www.unboundmedicine.com/medline/citation/32616563/Systemic_lupus_erythematosus_and_neutropaenia:_a_hallmark_of_haematological_manifestations L2 - https://lupus.bmj.com/cgi/pmidlookup?view=long&pmid=32616563 DB - PRIME DP - Unbound Medicine ER -
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