费尔蒂综合症,见解和更新。

Q4 Medicine
Open Rheumatology Journal Pub Date : 2014-12-31 eCollection Date: 2014-01-01 DOI:10.2174/1874312901408010129
Mohammad Bagher Owlia, Kam Newman, Mojtaba Akhtari
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引用次数: 45

摘要

Felty's综合征(FS)的特征是血清阳性的类风湿性关节炎(RA),伴有破坏性关节受累,脾肿大和中性粒细胞减少。目前的数据显示,1- 3%的RA患者合并FS,估计患病率为每10万人中有10人。完整的三联征不是绝对要求,但持续中性粒细胞减少,绝对中性粒细胞计数(ANC)通常小于1500/mm3是确定诊断的必要条件。费尔蒂综合征可能无症状,但严重的局部或全身感染可能是诊断的第一个线索。FS很容易被Sjӧgren综合征或系统性红斑狼疮或淋巴造血恶性肿瘤的平行诊断所忽视。与经典类风湿关节炎相比,遗传因子(HLA DR4)在FS中的作用更为突出。大量证据表明,在FS患者中,细胞免疫系统和体液免疫系统都参与了脾脏中性粒细胞的活化、凋亡及其对内皮细胞的粘附。研究表明,促炎细胞因子可能对骨髓颗粒生成有抑制作用。igg与中性粒细胞胞外染色质陷阱(NET)结合导致中性粒细胞死亡在其病理生理中起着至关重要的作用。反过来,“网状”中性粒细胞可能激活自身反应性B细胞,导致进一步的抗体和免疫复合物的形成。本文就Felty综合征的基本病理生理、流行病学、遗传学、临床、实验室及治疗进展等方面作一综述。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Felty's Syndrome, Insights and Updates.

Felty's Syndrome, Insights and Updates.

Felty's Syndrome, Insights and Updates.

Felty's Syndrome, Insights and Updates.

Felty's syndrome (FS) is characterized by the triad of seropositive rheumatoid arthritis (RA) with destructive joint involvement, splenomegaly and neutropenia. Current data shows that 1-3 % of RA patients are complicated with FS with an estimated prevalence of 10 per 100,000 populations. The complete triad is not an absolute requirement, but persistent neutropenia with an absolute neutrophil count (ANC) generally less than 1500/mm3 is necessary for establishing the diagnosis. Felty's syndrome may be asymptomatic but serious local or systemic infections may be the first clue to the diagnosis. FS is easily overlooked by parallel diagnoses of Sjӧgren syndrome or systemic lupus erythematosus or lymphohematopoietic malignancies. The role of genetic (HLA DR4) is more prominent in FS in comparison to classic rheumatoid arthritis. There is large body of evidence that in FS patients, both cellular and humoral immune systems participate in neutrophil activation, and apoptosis and its adherence to endothelial cells in the spleen. It has been demonstrated that proinflammatory cytokines may have inhibitory effects on bone marrow granulopoiesis. Binding of IgGs to neutrophil extracellular chromatin traps (NET) leading to neutrophil death plays a crucial role in its pathophysiology. In turn, "Netting" neutrophils may activate auto-reactive B cells leading to further antibody and immune complex formation. In this review we discuss on basic pathophysiology, epidemiology, genetics, clinical, laboratory and treatment updates of Felty's syndrome.

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来源期刊
Open Rheumatology Journal
Open Rheumatology Journal Medicine-Rheumatology
CiteScore
0.80
自引率
0.00%
发文量
2
期刊介绍: ENTHAM Open publishes a number of peer-reviewed, open access journals. These free-to-view online journals cover all major disciplines of science, medicine, technology and social sciences. BENTHAM Open provides researchers a platform to rapidly publish their research in a good-quality peer-reviewed journal. All peer-reviewed accepted submissions meeting high research and ethical standards are published with free access to all.
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