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21世纪的中性粒细胞减少症和费尔蒂综合征:重新定义类风湿关节炎患者的古老概念

Neutropenia and Felty Syndrome in the Twenty-First Century: Redefining Ancient Concepts in Rheumatoid Arthritis Patients.

作者信息

Rodas Flores Jorge Luis, Hernández-Cruz Blanca, Sánchez-Margalet Víctor, Fernández-Reboul Fernández Ana, Fernández Panadero Esther, Moral García Gracia, Pérez Venegas José Javier

机构信息

Rheumatology Department, Virgen Macarena University Hospital, Health Service of Andalucian, 41009 Seville, Spain.

Department of Medical Biochemistry and Molecular Biology and Immunology, School of Medicine, Virgen Macarena University Hospital, 41009 Seville, Spain.

出版信息

J Clin Med. 2024 Dec 17;13(24):7677. doi: 10.3390/jcm13247677.

Abstract

To describe the frequency of neutropenia and Felty syndrome in patients with rheumatoid arthritis (RA) attended in routine clinical practice. We selected by randomization a sample of 270 RA patients attended from January 2014 to November 2022. Demographic, clinical, and neutropenia-related variables were collected from the electronic medical records. Neutropenia was defined as having an absolute neutrophil count (ANC) of less than 1500/mm once, and acute if it persisted for <3 months. Felty syndrome was defined as RA-related neutropenia, rheumatoid factor (RF) and/or anti citrullinated protein antibody (ACPA) positivity. We found 50 patients who had at least one neutropenia episode, with an incidence of 18.5% (14.0-25.6%). Most were women, with age (mean, p25-p75) at the time of neutropenia of 61.5 (57.4-69.3) years, 85% RF+ and 76% ACPA+. The demographic and RA characteristics of patients with and without neutropenia were very similar, except for sex: most patients with neutropenia were women. The 50 patients had 99 episodes of neutropenia; 59% were acute. The lower ANC was 1240 (1000-1395) mm, and most of the episodes were mild (74%). In 32% of cases, there was other cytopenia. The RA activity measured by DAS28 in patients with neutropenia was low, at 2.18 (1.75-2.97). A total of 82 of 99 neutropenia episodes were related to DMARDs, 60% to Anti-IL6 drugs in monotherapy, 13% to RA activity, 3% to infectious diseases and 1% to hematologic malignancy. There were five (1.8%) cases with Felty syndrome, but only one woman with the classic combination of RA, positivity of autoantibodies (RF and ACPA), neutropenia and splenomegaly. In the 21st century, neutropenia in RA patients is most commonly related to biologics, mostly IL6 inhibitors and methotrexate. Episodes are mild, acute, with low RA activity, and associated with severe infections in few cases. Felty syndrome is rare.

摘要

描述在常规临床实践中类风湿关节炎(RA)患者中性粒细胞减少症和费尔蒂综合征的发生频率。我们通过随机化选择了2014年1月至2022年11月期间就诊的270例RA患者作为样本。从电子病历中收集人口统计学、临床和中性粒细胞减少症相关变量。中性粒细胞减少症定义为绝对中性粒细胞计数(ANC)一次低于1500/mm³,若持续时间<3个月则为急性中性粒细胞减少症。费尔蒂综合征定义为与RA相关的中性粒细胞减少症、类风湿因子(RF)和/或抗瓜氨酸化蛋白抗体(ACPA)阳性。我们发现50例患者至少有一次中性粒细胞减少症发作,发生率为18.5%(14.0 - 25.6%)。大多数为女性,中性粒细胞减少症发生时的年龄(中位数,第25 - 75百分位数)为61.5(57.4 - 69.3)岁,85%的患者RF阳性,76%的患者ACPA阳性。有中性粒细胞减少症和无中性粒细胞减少症患者的人口统计学和RA特征非常相似,除了性别:大多数有中性粒细胞减少症的患者为女性。这50例患者有99次中性粒细胞减少症发作;59%为急性发作。最低ANC为1240(1000 - 1395)/mm³,且大多数发作程度较轻(74%)。32%的病例存在其他血细胞减少症。中性粒细胞减少症患者中用DAS28测量的RA活动度较低,为2.18(1.75 - 2.97)。99次中性粒细胞减少症发作中共有82次与改善病情抗风湿药(DMARDs)相关,60%与单药使用的抗IL6药物相关,13%与RA活动度相关,3%与感染性疾病相关以及1%与血液系统恶性肿瘤相关。有5例(1.8%)患者患有费尔蒂综合征,但只有1名女性具备RA、自身抗体(RF和ACPA)阳性、中性粒细胞减少症和脾肿大的典型组合。在21世纪,RA患者的中性粒细胞减少症最常与生物制剂相关,主要是IL6抑制剂和甲氨蝶呤。发作程度较轻、为急性发作,RA活动度较低,且少数情况下与严重感染相关。费尔蒂综合征较为罕见。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/658b/11678567/0e90c7354529/jcm-13-07677-g001.jpg

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