Hur Minjun, Rattanathamsakul Natthapon, Jang Samuel, McClelland Collin M, Tajfirouz Deena A, Chodnicki Kevin D, McKeon Andrew, Pittock Sean J, Flanagan Eoin P, Zekeridou Anastasia, Chen John J
Departments of Ophthalmology (MH, NR, DAT, KDC, JJC), Neurology (NR, DAT, AM, SJP, EPF, AZ, JJC), and Radiology (SJ), Mayo Clinic, Rochester, Minnesota; and Department of Ophthalmology (CMM), University of Minnesota, Minneapolis, Minnesota.
J Neuroophthalmol. 2025 Jun 18. doi: 10.1097/WNO.0000000000002371.
Idiopathic cases of recurrent optic disc edema, which we termed idiopathic recurrent papillitis (IRP), share similar features with idiopathic recurrent neuroretinitis (IRN). This raises the possibility that they may be in the same spectrum of disease, in which long-term immunotherapies have been suggested to reduce relapses. We sought to characterize IRN and IRP, including the treatment effects of immunotherapy.
This retrospective, multicenter cohort study included patients with either IRN or papillitis (all without retrobulbar optic nerve enhancement on MRI during the acute attack) who were diagnosed at Mayo Clinic or University of Minnesota between January 2015 and October 2023. Demographics, age at first attack, and annualized relapse rates (ARRs) on and off long-term immunotherapy for patients on immunotherapy >6 months were recorded.
Among 30 patients, 17 (56.7%) patients had a neuroretinitis attack at least once, out of whom 10 (58.8%) patients had both neuroretinitis and papillitis attacks. Thirteen (43.3%) patients had only papillitis attacks. The median age of first attack was 34 years (interquartile range [IQR] 28-42) in neuroretinitis and 38 years (IQR 28-49) in papillitis (P = 0.91). The median number of attacks for all patients was 3.5 (IQR 3-5). Out of 15 patients who were on immunotherapy for 6 months or longer, 10 (66.7%) patients had at least 1 relapse with a median ARR on therapy of 0.28 attacks/year (IQR 0-0.54), which was not significantly different from off therapy (P = 0.74). Among immunotherapies, mycophenolate mofetil had the lowest median ARR (0 attacks/year, IQR 0-0.29), followed by intravenous immunoglobulin (0.49 attacks/year, IQR 0.25-0.74), methotrexate (0.49 attacks/year, IQR 0.29-0.87), rituximab/ocrelizumab (0.68 attacks/year, IQR 0.34-1.47), and azathioprine (0.75 attacks/year, IQR 0-2.17).
There is significant overlap between IRN and IRP, suggesting they are in the same spectrum of disease. Most long-term immunotherapies other than mycophenolate did not seem to be effective in reducing the number of relapses, although further prospective studies are required to assess this.
特发性复发性视盘水肿病例，我们称之为特发性复发性视乳头炎（IRP），与特发性复发性神经视网膜炎（IRN）具有相似特征。这增加了它们可能属于同一疾病谱系的可能性，其中有人提出长期免疫疗法可减少复发。我们试图对IRN和IRP进行特征描述，包括免疫疗法的治疗效果。
这项回顾性多中心队列研究纳入了2015年1月至2023年10月期间在梅奥诊所或明尼苏达大学被诊断为IRN或视乳头炎（急性发作期间MRI上均无球后视神经强化）的患者。记录了人口统计学信息、首次发作年龄以及接受免疫疗法超过6个月的患者在接受和未接受长期免疫疗法时的年化复发率（ARR）。
30例患者中，17例（56.7%）至少有一次神经视网膜炎发作，其中10例（58.8%）同时有神经视网膜炎和视乳头炎发作。13例（43.3%）患者仅有视乳头炎发作。神经视网膜炎首次发作的中位年龄为34岁（四分位间距[IQR]28 - 42），视乳头炎为38岁（IQR 28 - 49）（P = 0.91）。所有患者发作的中位次数为3.5次（IQR 3 - 5）。在15例接受免疫疗法6个月或更长时间的患者中，10例（66.7%）至少有1次复发，治疗期间的中位ARR为0.28次发作/年（IQR 0 - 0.54），与未治疗时无显著差异（P = 0.74）。在免疫疗法中，霉酚酸酯的中位ARR最低（0次发作/年，IQR 0 - 0.29），其次是静脉注射免疫球蛋白（0.49次发作/年，IQR 0.25 - 0.74）、甲氨蝶呤（0.49次发作/年，IQR 0.29 - 0.87）、利妥昔单抗/奥瑞珠单抗（（0.68次发作/年，IQR 0.34 - 1.47）和硫唑嘌呤（0.75次发作/年，IQR 0 - 2.17）。
IRN和IRP之间存在显著重叠，表明它们属于同一疾病谱系。除霉酚酸酯外，大多数长期免疫疗法似乎在减少复发次数方面无效，不过需要进一步的前瞻性研究来评估这一点。
