Fasano C, Bettiol A, Vastola M, Lotti A, Malandrino D, Mattioli I, Massacesi L, Emmi G, Barilaro A, Silvestri E
Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy.
Department of Neurology 2, Careggi University Hospital, 50134, Florence, Italy.
J Neurol. 2024 Dec 12;272(1):13. doi: 10.1007/s00415-024-12731-4.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a chronic inflammatory disease belonging to the spectrum of small-vessel vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCAs), also characterized by eosinophilic infiltration of target organs. Peripheral neuropathy (PN) affects about 2/3 of the patients as a presenting symptom and typically represents a vasculitic involvement. A few studies have addressed the role of intravenous immunoglobulin (IVIg) for the treatment of PN in EGPA. This monocentric retrospective study aims at assessing the effectiveness and safety of IVIg in patients with PN as the main acute manifestation at EGPA onset. The treatment with IVIg appears to be effective in inducing sustained remission, reducing the risk of relapses and improving the long-term disability due to its effects on PN.
嗜酸性肉芽肿性多血管炎（EGPA）是一种慢性炎症性疾病，属于与抗中性粒细胞胞浆抗体（ANCA）相关的小血管炎范畴，其特征还包括靶器官的嗜酸性粒细胞浸润。周围神经病变（PN）作为首发症状影响约2/3的患者，通常代表血管炎累及。一些研究探讨了静脉注射免疫球蛋白（IVIg）在EGPA中治疗PN的作用。这项单中心回顾性研究旨在评估IVIg对以PN为EGPA发病主要急性表现的患者的有效性和安全性。IVIg治疗似乎能有效诱导持续缓解，降低复发风险，并因其对PN的作用改善长期残疾状况。
