Akiyama Kaho, Iwasaki Yukiko, Tanaka Rie
Department of Ophthalmology, The University of Tokyo Hospital, Tokyo, Japan.
Department of Allergy and Rheumatology, The University of Tokyo Hospital, Tokyo, Japan.
Case Rep Ophthalmol. 2021 Jun 11;12(2):531-537. doi: 10.1159/000516851. eCollection 2021 May-Aug.
Adult Still's disease (ASD) is a rare systemic inflammatory disorder in which ocular manifestations have rarely been described. We report a 29-year-old Japanese woman with a rare case of refractory ASD complicated by Purtscher-like retinopathy. She was diagnosed with ASD and started on a high dose of oral prednisolone. Two days after the initiation of the treatment, she presented with blurred vision in the left eye, and the funduscopic examination revealed bilateral Purtscher-like retinopathy. Despite treatment with high-dose oral prednisolone for 2 weeks, she developed macrophage activation syndrome. Considering the severity of ASD, intravenous pulse methylprednisolone therapy and tocilizumab injection were administered. Although all the laboratory data and Purtscher-like retinopathy gradually improved, nerve fiber layer defect (NFLD) in both eyes appeared and visual field defect remained corresponding to the NFLD. In conclusion, Purtscher-like retinopathy might be useful as a poor prognostic factor of ASD, which needs appropriate systemic immunosuppressive treatment. Early detection and long-term follow-up of Purtscher-like retinopathy is important because it has the possibility of developing permanent visual field defect.
成人斯蒂尔病（ASD）是一种罕见的全身性炎症性疾病，眼部表现鲜有报道。我们报告了一例29岁的日本女性，患有罕见的难治性ASD并并发类Purtscher样视网膜病变。她被诊断为ASD并开始服用高剂量口服泼尼松龙。治疗开始两天后，她出现左眼视力模糊，眼底检查发现双侧类Purtscher样视网膜病变。尽管使用高剂量口服泼尼松龙治疗2周，她仍发展为巨噬细胞活化综合征。考虑到ASD的严重性，给予静脉注射甲基泼尼松龙脉冲疗法和托珠单抗注射。尽管所有实验室数据和类Purtscher样视网膜病变逐渐改善，但双眼出现神经纤维层缺损（NFLD），视野缺损仍与NFLD相对应。总之，类Purtscher样视网膜病变可能是ASD预后不良的一个因素，需要适当的全身免疫抑制治疗。对类Purtscher样视网膜病变进行早期检测和长期随访很重要，因为它有可能发展为永久性视野缺损。
