Gorostis S, Bacha M, Gravier S, Raguin T
Service d'ORL et chirurgie cervico-faciale, CHU de Hautepierre, avenue Molière, 67200 Strasbourg, France.
3 bis, rue de la Semm, 68000 Colmar, France.
Eur Ann Otorhinolaryngol Head Neck Dis. 2017 Oct;134(5):351-354. doi: 10.1016/j.anorl.2017.02.012. Epub 2017 Mar 27.
Eosinophilic angiocentric fibrosis (EAF) is a slowly progressive, benign disease involving the mucosa of the upper airways or, more rarely, the orbit. It belongs to the spectrum of IgG4-related disease.
The authors report the case of a 61-year-old man who presented with orbital involvement (visual loss, pain, proptosis, and eyelid oedema), headache and nasal obstruction. Imaging revealed a right ethmoido-orbital mass infiltrating the periorbital fat and enveloping the optic nerve. Histological examination concluded on a diagnosis of EAF in the presence of perivascular infiltration by inflammatory cells, predominantly eosinophils, and zones of "onion skin" fibrosis. Immunohistochemistry attributed these lesions to IgG4-related disease. Initial treatment with corticosteroids followed by dapsone failed to control the disease and resulted in severe steroid dependence. Surgical ethmoidectomy with resection of the lamina papyracea was performed to allow displacement of the eyeball into the nasal cavity in the event of another episode.
The combination of surgery and rituximab achieved lasting pain relief with no recurrence of exophthalmos.
嗜酸性粒细胞性血管中心性纤维化（EAF）是一种缓慢进展的良性疾病，累及上呼吸道黏膜，或更罕见地累及眼眶。它属于IgG4相关性疾病范畴。
作者报告了一例61岁男性患者，其表现为眼眶受累（视力丧失、疼痛、眼球突出和眼睑水肿）、头痛和鼻塞。影像学检查显示右侧筛窦-眼眶肿物，浸润眶周脂肪并包绕视神经。组织学检查在存在以嗜酸性粒细胞为主的炎性细胞血管周围浸润及“洋葱皮”样纤维化区域的情况下诊断为EAF。免疫组织化学将这些病变归因于IgG4相关性疾病。最初使用皮质类固醇治疗，随后使用氨苯砜，未能控制病情，导致严重的类固醇依赖。进行了筛窦切除术并切除纸样板，以便在再次发作时使眼球移位至鼻腔。
手术与利妥昔单抗联合使用实现了持久的疼痛缓解，眼球突出未复发。
