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甲磺酸伊马替尼治疗难治性嗜酸性肉芽肿伴多血管炎:文献回顾及病例报告。

Imatinib mesylate use in refractory eosinophilic granulomatosis with polyangiitis: a literature review and a case report.

机构信息

Department of Autoimmune Disorders in Rheumatic Diseases, V.A. Nasonova Research Institute of Rheumatology, Kashirskoe shosse 34A, Moscow, Russian Federation, 115522.

Department of Rheumaorthopedics and Rehabilitation, V.A. Nasonova Research Institute of Rheumatology, Kashirskoe shosse 34A, Moscow, Russian Federation, 115522.

出版信息

Clin Rheumatol. 2018 Jun;37(6):1729-1735. doi: 10.1007/s10067-018-4018-1. Epub 2018 Mar 21.

Abstract

Recent advances in pharmacology have greatly expanded the drug repertoire for treatment of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic disorder, a type of the ANCA-associated vasculitis. Important features of this disease are eosinophilia and anti-myeloperoxidase ANCA presence in around 30-70% of patients. Primary therapy of EGPA includes steroids and cytotoxic drugs, e.g., cyclophosphamide, azathioprine, or methotrexate. Nevertheless, some patients are refractory to this therapy. Alternative approaches include rituximab, mepolizumab, and intravenous immunoglobulin. Accumulating evidence highlight a new promising drug in EGPA therapy-imatinib mesylate (IM), tyrosine kinase inhibitor. This drug is a key pharmacological agent in treating various types of hematological malignancies and FIP1L1/PDGF-RA-positive hypereosinophilia. In this article, we present a case demonstrating successful treatment of EGPA with IM; we also discuss possible mechanisms of IM efficacy in EGPA treatment and future perspectives of this therapeutic approach.

摘要

近年来,药理学的进展极大地扩展了治疗抗中性粒细胞胞质抗体(ANCA)相关性血管炎的药物种类。嗜酸性肉芽肿伴多血管炎(EGPA)是一种罕见的多系统疾病,属于 ANCA 相关性血管炎的一种类型。这种疾病的重要特征是大约 30-70%的患者存在嗜酸性粒细胞增多和抗髓过氧化物酶 ANCA。EGPA 的主要治疗方法包括类固醇和细胞毒性药物,如环磷酰胺、硫唑嘌呤或甲氨蝶呤。然而,一些患者对此治疗方法有抗药性。替代方法包括利妥昔单抗、美泊利珠单抗和静脉注射免疫球蛋白。越来越多的证据表明,伊马替尼甲磺酸盐(IM),一种酪氨酸激酶抑制剂,是 EGPA 治疗中一种有前途的新药。这种药物是治疗各种类型血液学恶性肿瘤和 FIP1L1/PDGF-RA 阳性嗜酸性粒细胞增多症的关键药物。本文介绍了一例用 IM 成功治疗 EGPA 的病例,并讨论了 IM 在 EGPA 治疗中的可能作用机制及该治疗方法的未来前景。

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