Systemic lupus erythematosus and neutropaenia: a hallmark of haematological manifestations.

Autor:	Meyer A; 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., Guffroy A; 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., Blaison G; Department of Internal Medicine, Hôpitaux Civils de Colmar, Colmar, Alsace, France., Dieudonne Y; 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., Amoura Z; Department of Internal Medicine, Institut E3M, Assistance Publique Hôpitaux de Paris (APHP), Groupement Hospitalier Pitié Salpêtrière, Paris, France., Bonnotte B; Department of Clinical Immunology and Internal Medicine, CHU Dijon Bourgogne, Dijon, France., Fiehn C; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany., Kieffer P; Department of Internal Medicine, Centre hospitalier de Mulhouse, Mulhouse, France., Lorenz HM; Department of Medicine V, University Hospital Heidelberg, Center for Rheumatic Diseases Baden-Baden, Heidelberg, Germany., Magy-Bertrand N; Department of Internal Medicine, CHU de Besançon, Besançon, France., Maurier F; Department of Internal Medicine, Hôpitaux Privés de Metz, Metz, France., Pennaforte JL; Department of Internal Medicine, CHU de Reims, Hôpital Robert Debré, Reims, France., Peter HH; Department of Rheumatology and Clinical Immunology, Medical Center - Faculty of Medicine, University of Freiburg, Freiburg, Germany., Schwarting A; Department of Internal Medicine, Universitätsmedizin, Mainz, Germany., Sibilia J; Department of Rheumatology, National Reference Center for autoimmune diseases (RESO), Hôpitaux universitaires de Strasbourg, Strasbourg, France., Arnaud L; 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., Martin T; 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., Voll RE; Department of Rheumatology and Clinical Immunology, Medical Center - Faculty of Medicine, University of Freiburg, Freiburg, Germany., Korganow AS; 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.
Jazyk:	angličtina
Zdroj:	Lupus science & medicine [Lupus Sci Med] 2020 Jul; Vol. 7 (1).
DOI:	10.1136/lupus-2020-000399
Abstrakt:	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 1800×10 6 /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. Competing Interests: Competing interests: None declared. (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)
Databáze:	MEDLINE
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