Departments of Rheumatology (JAU, IA, CMW), Ophthalmology (JW, YJL), and Pathology (GJB), Stanford University School of Medicine, Stanford, California; and Department of Rheumatology (US), Sequoia Hospital, Redwood City, California.
J Neuroophthalmol. 2018 Mar;38(1):17-23. doi: 10.1097/WNO.0000000000000566.
We describe a 75-year-old woman who experienced vision loss in her left eye due to biopsy-proven giant cell arteritis (GCA). She subsequently developed pachymeningitis causing refractory headaches and bilateral optic neuropathy and maculopathy.
Case report with literature review.
Eighteen months after the initial diagnosis of GCA, imaging studies in our patient demonstrated pachymeningeal enhancement, and meningeal biopsy confirmed lymphoplasmacytic tissue infiltrates with low frequencies of IgG4+ plasma cells. Laboratory investigation revealed the presence of 3 antiretinal antibodies and antimyeloperoxidase antibodies, consistent with autoimmune retinopathy. Treatment with B-cell-depleting anti-CD20 antibodies suppressed meningeal inflammation and prevented further vision loss.
This case illustrates that bilateral vision loss and chronic headaches in patients with GCA may result from retina-directed autoimmunity and pachymeningitis.
我们描述了一位 75 岁女性，她因活检证实的巨细胞动脉炎（GCA）左眼视力丧失。随后发生了导致难治性头痛和双侧视神经病变和黄斑病变的硬脑膜炎。
病例报告并文献复习。
在 GCA 的初始诊断后 18 个月，我们患者的影像学研究显示硬脑膜增强，脑膜活检证实了淋巴浆细胞组织浸润，IgG4+浆细胞频率较低。实验室检查显示存在 3 种抗视网膜抗体和抗髓过氧化物酶抗体，符合自身免疫性视网膜炎。用 B 细胞耗竭性抗 CD20 抗体抑制脑膜炎症，防止进一步的视力丧失。
本例说明 GCA 患者的双侧视力丧失和慢性头痛可能是由视网膜定向自身免疫和硬脑膜炎引起的。
