Isozaki Takeo, Homma Tetsuya, Sagara Hironori, Kasama Tsuyoshi
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.
J Clin Med. 2020 Nov 30;9(12):3890. doi: 10.3390/jcm9123890.
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.
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。