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.
Lupus Sci Med. 2020 Jul;7(1). doi: 10.1136/lupus-2020-000399.
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.
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/L. An additional analysis of detailed medical records was done for 65 LBBR patients with neutropaenia.
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.
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.
系统性红斑狼疮是一种慢性自身免疫性疾病，其表型具有异质性。中性粒细胞减少症是 SLE 的常见事件，根据中性粒细胞计数的阈值，有 20%-40%的患者发生中性粒细胞减少症。在日常实践中，SLE 中性粒细胞减少症的管理存在多种可能的原因，较为困难。此外，SLE 中性粒细胞减少症的感染后果仍未得到明确界定。
本研究纳入了来自上莱茵狼疮生物库（LBBR）的 998 名患者，这是一个大型的德国和法国 SLE 患者队列，大多数为白种人（83%）。当中性粒细胞计数低于 1800×10/L 时，考虑存在中性粒细胞减少症。对 LBBR 中性粒细胞减少症的 65 名患者的详细病历进行了额外分析。
与 779 名无中性粒细胞减少症的 SLE 患者相比，208 名中性粒细胞减少症患者（21%）。在多变量分析中，SLE 中性粒细胞减少症与血小板减少症（OR 4.11（2.57-10.3））、淋巴细胞减少症（OR 4.41（2.51-11.5））和低 C3（OR 1.91（1.03-4.37））显著相关。对 65 名具有代表性的中性粒细胞减少症患者进行了分析。中性粒细胞减少症为中重度的占 38%，慢性的占 31%，中重度和慢性的占 23%。中重度和慢性中性粒细胞减少症均与淋巴细胞减少症和血小板减少症相关。慢性中性粒细胞减少症还与抗 Ro/SSA 抗体相关，而中重度中性粒细胞减少症与口腔溃疡相关。
本研究是迄今为止描述 SLE 中性粒细胞减少症的最大队列。中性粒细胞减少症与其他细胞减少症有很强的关联，提示存在共同的机制。慢性中性粒细胞减少症与抗 Ro/SSA 抗体相关，无论是否存在已确诊的干燥综合征。
