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Eosinophilic Granulomatosis With Polyangiitis: Clinical Predictors of Long-term Asthma Severity.嗜酸性肉芽肿性多血管炎:长期哮喘严重程度的临床预测因素。
Chest. 2020 May;157(5):1086-1099. doi: 10.1016/j.chest.2019.11.045. Epub 2020 Jan 17.
2
Eosinophilic granulomatosis with polyangiitis: the multifaceted spectrum of clinical manifestations at different stages of the disease.嗜酸性肉芽肿性多血管炎:疾病不同阶段临床表现的多面谱。
Expert Rev Clin Immunol. 2020 Jan;16(1):51-61. doi: 10.1080/1744666X.2019.1697678. Epub 2020 Jan 17.
3
Destructive Upper Airway Disease from Eosinophilic Granulomatosis with Polyangiitis (EGPA): The Very First Case.嗜酸性肉芽肿性多血管炎(EGPA)所致的破坏性上气道疾病:首例病例
Case Rep Rheumatol. 2019 May 23;2019:6173869. doi: 10.1155/2019/6173869. eCollection 2019.
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Safety and Efficacy of Mepolizumab in Patients with Eosinophilic Granulomatosis with Polyangiitis.美泊利单抗治疗嗜酸性肉芽肿性多血管炎患者的安全性和有效性
Pulm Med. 2019 Mar 3;2019:4376380. doi: 10.1155/2019/4376380. eCollection 2019.
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Serum cytokine and chemokine levels in patients with eosinophilic granulomatosis with polyangiitis, hypereosinophilic syndrome, or eosinophilic asthma.嗜酸粒细胞性肉芽肿性多血管炎、高嗜酸性粒细胞综合征或嗜酸细胞性哮喘患者的血清细胞因子和趋化因子水平。
Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):40-44. Epub 2019 Jan 14.
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Serum periostin as a biomarker in eosinophilic granulomatosis with polyangiitis.血清骨膜蛋白作为嗜酸性肉芽肿伴多血管炎的生物标志物。
PLoS One. 2018 Oct 11;13(10):e0205768. doi: 10.1371/journal.pone.0205768. eCollection 2018.
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Severe/uncontrolled asthma and overall survival in atopic patients with eosinophilic granulomatosis with polyangiitis.变应性肉芽肿性多血管炎伴嗜酸性粒细胞增多症患者中严重/未控制的哮喘与总生存。
Respir Med. 2018 Sep;142:66-72. doi: 10.1016/j.rmed.2018.07.017. Epub 2018 Jul 24.
8
Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis.美泊利单抗或安慰剂用于嗜酸性肉芽肿性多血管炎
N Engl J Med. 2017 May 18;376(20):1921-1932. doi: 10.1056/NEJMoa1702079.
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Role of IL-13 Genetic Variants in Signalling of Asthma.白细胞介素-13 遗传变异在哮喘信号转导中的作用。
Inflammation. 2017 Apr;40(2):566-577. doi: 10.1007/s10753-016-0503-3.
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Am J Respir Cell Mol Biol. 2016 Nov;55(5):675-683. doi: 10.1165/rcmb.2016-0099OC.

细胞因子在嗜酸性粒细胞肉芽肿性多血管炎中的作用及抗白细胞介素-5抗体治疗的可能性

Role of Cytokines in EGPA and the Possibility of Treatment with an Anti-IL-5 Antibody.

作者信息

Isozaki Takeo, Homma Tetsuya, Sagara Hironori, Kasama Tsuyoshi

机构信息

Division of Rheumatology, Department of Medicine, Showa University School of Medicine, Tokyo 142-8666, Japan.

Division of Respiratory Medicine and Allergology, Department of Medicine, Showa University School of Medicine, Tokyo 142-8666, Japan.

出版信息

J Clin Med. 2020 Nov 30;9(12):3890. doi: 10.3390/jcm9123890.

DOI:10.3390/jcm9123890
PMID:33265990
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7760889/
Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of systemic vasculitis with eosinophilia in the peripheral blood, which is preceded by bronchial asthma or allergic disease. EGPA is pathologically characterized by microangiopathy granulomatosis vasculitis. Vasculitis can be exacerbated and cause central nervous system and cardiovascular disorders and gastrointestinal perforation. Histological examination reveals eosinophil infiltration and granulomas in lesions in areas such as the lung, nervous system, and skin. Laboratory tests show inflammatory findings such as C-reactive protein (CRP) elevation, increased eosinophils, elevated serum IgE, and elevated myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPO-ANCA). MPO-ANCA is positive in approximately 40-70% of cases of this disease. EGPA is a necrotizing vasculitis that affects small- and medium-sized blood vessels; however, it differs from other types of ANCA-related vasculitis (such as microscopic polyangiitis and granulomatosis) because it is preceded by bronchial asthma and eosinophilia in the blood and tissues. Treatment with immunosuppressive agents such as steroids or cyclophosphamide depends on the Five Factor Score, which predicts the prognosis and severity of the condition. If the effect of appropriate treatment with steroids is insufficient, the anti-interleukin-5 antibody mepolizumab can be administered. The combination of mepolizumab with standard treatment leads to a significantly longer duration of remission, a higher proportion of patients who achieve sustained remission, and less steroid use than with a placebo.

摘要

嗜酸性肉芽肿性多血管炎(EGPA)是一种外周血嗜酸性粒细胞增多的系统性血管炎,其发病前常有支气管哮喘或过敏性疾病。EGPA的病理特征为微血管病性肉芽肿性血管炎。血管炎可加重并导致中枢神经系统和心血管系统疾病以及胃肠道穿孔。组织学检查显示在肺、神经系统和皮肤等部位的病变中有嗜酸性粒细胞浸润和肉芽肿形成。实验室检查显示炎症表现,如C反应蛋白(CRP)升高、嗜酸性粒细胞增多、血清IgE升高以及髓过氧化物酶抗中性粒细胞胞浆抗体(MPO-ANCA)升高。在本病约40%-70%的病例中MPO-ANCA呈阳性。EGPA是一种累及中小血管的坏死性血管炎;然而,它与其他类型的ANCA相关血管炎(如显微镜下多血管炎和肉芽肿性血管炎)不同,因为它发病前有支气管哮喘以及血液和组织中的嗜酸性粒细胞增多。使用类固醇或环磷酰胺等免疫抑制剂进行治疗取决于五因素评分,该评分可预测病情的预后和严重程度。如果类固醇的适当治疗效果不佳,可以使用抗白细胞介素-5抗体美泊利单抗。与安慰剂相比,美泊利单抗与标准治疗联合使用可显著延长缓解期,使更多患者实现持续缓解,并减少类固醇的使用。