Gilyarov M Yu, Belikova L P, Shchukin I A, Fidler M S, Avakyan G G, Shustrova G E, Martynov M Yu, Svet A V
Pirogov City Clinical Hospital, Moscow, Russia.
Pirogov City Clinical Hospital, Moscow, Russia; Pirogov Russian National Research Medical University, Moscow, Russia.
Zh Nevrol Psikhiatr Im S S Korsakova. 2016;116(10):93-102. doi: 10.17116/jnevro201611610193-102.
Eosinophilic granulomatosis with polyangiitis - EGPA (Churg-Strauss syndrome) is a rare autoimmune disorder. The pathogenesis of the disease includes production of anti-neutrophil cytoplasmic antibodies directed against myeloperoxidase with the development of small-vessel necrotizing vasculitis and eosinophilic infiltration of organs. The involvement of peripheral and central nervous system is observed in more than 3/4 of cases. The authors describe three patients with EGPA. In a 53-year-old male patient, EGPA manifested with multiple neuropathies, which regressed after treatment with corticosteroids and cytostatics. In a 34-year-old woman, cerebral sinus thrombosis and cerebral infarction developed in the non-active period of long-term EGPA. The patient was treated with anticoagulants. A 77-year-old woman with a newly diagnosed EGPA, confirmed by bone marrow examination for eosinophilia, developed ischemic stroke and polyneuropathy. The causes and mechanisms of development as well as dynamics and outcomes of neurological disorders, differential diagnosis, treatment and prognosis of eosinophilic granulomatosis with polyangiitis are discussed.
嗜酸性肉芽肿性多血管炎（EGPA，又称变应性肉芽肿性血管炎）是一种罕见的自身免疫性疾病。该病的发病机制包括产生针对髓过氧化物酶的抗中性粒细胞胞浆抗体，进而发展为小血管坏死性血管炎以及器官的嗜酸性粒细胞浸润。超过3/4的病例会出现外周和中枢神经系统受累。作者描述了3例EGPA患者。一名53岁男性患者，EGPA表现为多发性神经病，经皮质类固醇和细胞毒性药物治疗后病情好转。一名34岁女性，在长期EGPA的非活动期发生了脑静脉窦血栓形成和脑梗死，该患者接受了抗凝治疗。一名77岁女性经骨髓检查证实嗜酸性粒细胞增多，新诊断为EGPA，发生了缺血性中风和多发性神经病。本文讨论了嗜酸性肉芽肿性多血管炎神经系统疾病的发生原因和机制、动态变化及转归、鉴别诊断、治疗和预后。
