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费尔蒂综合征和系统性红斑狼疮中自身免疫性中性粒细胞减少症的治疗。

Management of autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus.

机构信息

Department of Internal Medicine, Jamaica Hospital Medical Center, NY 11418, United States.

出版信息

Autoimmun Rev. 2011 May;10(7):432-7. doi: 10.1016/j.autrev.2011.01.006. Epub 2011 Jan 18.

Abstract

Autoimmune neutropenia, caused by neutrophil-specific autoantibodies is a common phenomenon in autoimmune disorders such as Felty's syndrome and systemic lupus erythematosus. Felty's syndrome is associated with neutropenia and splenomegaly in seropositive rheumatoid arthritis which can be severe and with recurrent bacterial infections. Neutropenia is also common in systemic lupus erythematosus and it is included in the current systemic lupus classification criteria. The pathobiology of the autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus is complex, and it could be a major cause of morbidity and mortality due to increased risk of sepsis. Treatment should be individualized on the basis of patient's clinical situation, and prevention or treatment of the infection. Recombinant human granulocyte colony-stimulating factor is a safe and effective therapeutic modality in management of autoimmune neutropenia associated with Felty's syndrome and systemic lupus erythematosus, which stimulates neutrophil production. There is a slight increased risk of exacerbation of the underlying autoimmune disorder, and recombinant human granulocyte colony-stimulating factor dose and frequency should be adjusted at the lowest effective dose.

摘要

自身免疫性中性粒细胞减少症由中性粒细胞特异性自身抗体引起,是自身免疫性疾病(如费尔蒂综合征和系统性红斑狼疮)中的常见现象。费尔蒂综合征与类风湿关节炎的中性粒细胞减少症和脾肿大相关,这种情况可能很严重,且易发生反复细菌感染。中性粒细胞减少症在系统性红斑狼疮中也很常见,它被包含在当前的系统性红斑狼疮分类标准中。费尔蒂综合征和系统性红斑狼疮自身免疫性中性粒细胞减少症的发病机制很复杂,它可能是由于败血症风险增加导致发病率和死亡率增加的主要原因。应根据患者的临床情况进行个体化治疗,并预防或治疗感染。重组人粒细胞集落刺激因子是治疗与费尔蒂综合征和系统性红斑狼疮相关的自身免疫性中性粒细胞减少症的安全有效治疗方法,它可刺激中性粒细胞生成。这种治疗方法可能会略微增加潜在自身免疫性疾病恶化的风险,应调整重组人粒细胞集落刺激因子的剂量和频率,以最低有效剂量进行治疗。

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