Multi-targeted therapy for refractory eosinophilic granulomatosis with polyangiitis characterized by intracerebral hemorrhage and cardiomyopathy: a case-based review
Multi-targeted therapy for refractory eosinophilic granulomatosis with polyangiitis characterized by intracerebral hemorrhage and cardiomyopathy: a case-based review
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以脑出血和心肌病为特征的难治性嗜酸性肉芽肿伴多血管炎的多靶点治疗：基于病例的回顾
DOI：
10.1007/s00296-021-04950-z
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发表时间：
2021
影响因子：
4
通讯作者：
Harigae Hideo
中科院分区：
文献类型：
--
作者：
Mutoh Tomoyuki;Shirai Tsuyoshi;Sato Hiroko;Fujii Hiroshi;Ishii Tomonori;Harigae Hideo
嗜酸性肉芽肿病合并多血管炎（EGPA）是一种系统性自身免疫性疾病，属于抗中性粒细胞细胞质抗体（ANCA）相关血管炎，主要影响中小血管，以哮喘、嗜酸性粒细胞增多和坏死性肉芽肿性炎症为特征。大多数EGPA患者表现为周围神经病变，而脑出血因EGPA相关表现累及中枢神经系统而罕见，导致严重的发病率和死亡率。在这里，我们提出一个45岁的男性顽固性EGPA，以脑出血为首发表现，其次是心脏受累。有支气管哮喘病史的患者发生EGPA引起的右侧皮膜出血。尽管静脉注射环磷酰胺（IVCY）和美polizumab （MPZ）诱导缓解，但经常观察到复发。随后，尽管使用利妥昔单抗（RTX）代替IVCY和MPZ，患者仍出现心肌病。需要联合免疫抑制治疗，包括IVCY， MPZ和RTX来抑制血管炎症，导致持续缓解。我们在回顾以往发表的文献的同时，重点关注EGPA脑出血患者的临床特点，描述脑出血患者检测EGPA的临床特点，强调在该病的早期血管期快速评估和识别EGPA并进行充分的干预。我们也提到这个病例的免疫学方面。在难治性EGPA的治疗中，考虑通过白细胞介素-5抑制和B细胞消耗的“多靶向治疗”是很重要的。
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic autoimmune disorder classified under anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, predominantly affecting small- to medium-sized vessels, characterized by asthma, eosinophilia, and necrotizing granulomatous inflammation. Most patients with EGPA experience peripheral neuropathy, whereas intracerebral hemorrhage is rare as EGPA-related presentation in central nervous system involvement, causing severe morbidity and mortality. Here, we present a 45-year-old man with refractory EGPA who developed intracerebral hemorrhage as the first manifestation, followed by cardiac involvement. This patient with a history of bronchial asthma developed a right putaminal hemorrhage caused by EGPA. Although intravenous cyclophosphamide (IVCY) and mepolizumab (MPZ) induced remission, relapse was frequently observed. Subsequently, he developed cardiomyopathy despite administration of rituximab (RTX) substituted from IVCY and MPZ. Combined immunosuppressive therapy, including IVCY, MPZ, and RTX was required to inhibit vascular inflammation, leading to sustained remission. We review previously published literature while focusing on the clinical features of patients with intracerebral hemorrhage caused by EGPA and describe clinical characteristics for detecting EGPA in patients with intracerebral hemorrhage, emphasizing rapid evaluation and recognition of EGPA and adequate intervention in the early vasculitic phase of this disease. We also refer to the immunological aspects of this case. It is important to consider “multi-targeted therapy” through interleukin-5 suppression and B cell depletion in the management of refractory EGPA.
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影响因子：
5.1
作者：
Wolf, J.;Bergner, R.;Grau, A. J.
通讯作者：
Grau, A. J.
脑内出血，Churg-Strauss 综合征的可能表现：病例报告和文献综述
影响因子：
4.4
作者：
N. Mencacci;A. Bersano;C. Cinnante;A. Ciammola;S. Corti;Marcus John Beasley;V. Silani
通讯作者：
V. Silani
影响因子：
6.2
作者：
Teixeira, Vitor;Mohammad, Aladdin J.;Jayne, David
通讯作者：
Jayne, David
伴有多发性脑出血的嗜酸性肉芽肿性多血管炎——病例报告。
DOI：
--
发表时间：
2016
期刊：
JPMA. The Journal of the Pakistan Medical Association
影响因子：
--
作者：
Z. Ullah;Z. Abideen;R. Shoaib;Arsalan Ahmad;Q. Zaman;R. Nazir
通讯作者：
R. Nazir
影响因子：
--
作者：
Jennette, J. C.;Falk, R. J.;Watts, R. A.
通讯作者：
Watts, R. A.