1. Systemic lupus erythematosus and neutropaenia: a hallmark of haematological manifestations
- Author
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Aurore, Meyer, Aurélien, Guffroy, Gilles, Blaison, Yannick, Dieudonne, Zahir, Amoura, Bernard, Bonnotte, Christoph, Fiehn, Pierre, Kieffer, Hannes Martin, Lorenz, Nadine, Magy-Bertrand, François, Maurier, Jean-Louis, Pennaforte, Hans-Hartmut, Peter, Andreas, Schwarting, Jean, Sibilia, Laurent, Arnaud, Thierry, Martin, Reinhard Edmund, Voll, Anne-Sophie, Korganow, R, Voll, CHU Strasbourg, Université de Strasbourg (UNISTRA), Immuno-Rhumatologie Moléculaire, Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Médecine interne [Hôpitaux civils de Colmar], Hôpitaux Civils de Colmar, Institut E3M [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier Emile Muller [Mulhouse] (CH E.Muller Mulhouse), Groupe Hospitalier de Territoire Haute Alsace (GHTHA), Heidelberg University Hospital [Heidelberg], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Hôpitaux Privés de Metz (HPMetz), Centre Hospitalier Universitaire de Reims (CHU Reims), University of Freiburg [Freiburg], Les Hôpitaux Universitaires de Strasbourg (HUS), and LBBR/Rarenet group: Z Amoura, L Arnaud, G Blaison, B Bonnotte, E Chatelus, E Ciobanu, F Duchene, J P Faller, A Gorse, J E Gottenberg, O Hinschberger, F Jaeger, P Kieffer, M Kilifa, N Magy-Bertrand, T Martin, L Martzolff, F Maurier, A Meyer, J-L Pasquali, J-L Pennaforte, V Poindron, S Revuz, M Samson, J Sibilia, C Sordet, A Theulin, D Wahl, J C Weber, M Bartsch, N Bartholomä, C Fiehn, S Finzel, A Funkert, M Hausberg, H Lorenz, R Max, H-H Peter, M Rizzi, A Schwarting, J Thiel, N Venhoff, R Voll
- Subjects
Adult ,Male ,0301 basic medicine ,medicine.medical_specialty ,Neutropenia ,autoantibodies ,Immunology ,Disease ,Sciences du Vivant [q-bio]/Médecine humaine et pathologie ,Autoimmune Diseases ,03 medical and health sciences ,0302 clinical medicine ,Germany ,Lymphopenia ,lupus erythematosus, systemic ,Internal medicine ,medicine ,Humans ,Oral ulcers ,030203 arthritis & rheumatology ,Autoimmune disease ,Systemic lupus erythematosus ,Genetic heterogeneity ,business.industry ,Autoantibody ,Complement C3 ,General Medicine ,systemic ,medicine.disease ,Thrombocytopenia ,Epidemiology and Outcomes ,3. Good health ,Cross-Sectional Studies ,Sjogren's Syndrome ,030104 developmental biology ,Antibodies, Antinuclear ,Case-Control Studies ,Cohort ,Absolute neutrophil count ,Female ,France ,business ,lupus erythematosus ,[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology - Abstract
ObjectiveSystemic 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.Methods998 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.Results208 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.ConclusionThis 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.
- Published
- 2020
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