Ege University Faculty of Medicine, Department of Ophthalmology, İzmir, Türkiye.
Ege University Faculty of Medicine, Department of Pathology, İzmir, Türkiye.
Turk J Ophthalmol. 2023 Apr 20;53(2):79-84. doi: 10.4274/tjo.galenos.2022.34683.
To evaluate the demographic data, ocular and systemic findings, clinical management, and outcomes of patients with ocular cicatricial pemphigoid (OCP).
The medical records of 11 patients diagnosed as having OCP in the ophthalmology department of Ege University between 2008 and 2021 were evaluated retrospectively.
The patients' mean follow-up time was 14±5.76 months. All eyes (100%) had conjunctival involvement and 18 (81.81%) had corneal involvement. According to the Tauber staging system, 7 (31.81%), 8 (36.36%), and 7 (31.81%) of the eyes were stage 2, 3, and 4, respectively. The diagnosis was confirmed in 6 (66.66%) of 9 patients who underwent biopsy. Amniotic membrane transplantation was performed in 7 eyes, entropion surgery in 2 eyes, and electrocauterization for trichiasis in 5 eyes. Systemic involvement was observed in 45.45% (5/11) of patients, most commonly oral mucosal involvement (18.18%). Review of medical records showed that alkylating agents, steroids, and dapsone were used in patients treated before 2020. Mycophenolate mofetil was preferred to be used in combination with corticosteroids. Although treatment responses before mycophenolate mofetil usage could not be evaluated well because of loss to follow-up, 4 (66.66%) of 6 patients who received steroid treatment combined with mycophenolate mofetil showed partial or complete clinical remission. No serious side effects and drug withdrawal were observed.
OCP is a sight-threatening autoimmune disease that affects older adults. Although positive biopsy results are valuable for diagnosis, negative results do not exclude the diagnosis. The main treatment is systemic immunosuppressives. Disease activity can be suppressed, especially with early initiation of drug therapy. These patients require a multidisciplinary approach. Especially in the presence of isolated ocular findings, ophthalmologists should be able to make the decision to start immunosuppressive treatment, and systemic treatment should not be delayed.
评估眼瘢痕性类天疱疮（OCP）患者的人口统计学数据、眼部和全身表现、临床管理和结局。
回顾性分析 2008 年至 2021 年期间在伊兹密尔大学眼科部门被诊断为 OCP 的 11 例患者的病历。
患者的平均随访时间为 14±5.76 个月。所有眼睛（100%）均有结膜受累，18 只（81.81%）有角膜受累。根据 Tauber 分期系统，2 只（31.81%）、3 只（36.36%）和 4 只（31.81%）眼分别为 2 期、3 期和 4 期。9 例患者中有 6 例（66.66%）进行了活检以确认诊断。7 只眼行羊膜移植，2 只眼行内翻矫正术，5 只眼行电凝治疗倒睫。45.45%（5/11）的患者有全身受累，最常见的是口腔黏膜受累（18.18%）。回顾病历发现，2020 年前接受治疗的患者使用过烷化剂、类固醇和氨苯砜。更倾向于使用霉酚酸酯联合皮质类固醇。虽然由于失访无法很好地评估霉酚酸酯使用前的治疗反应，但 6 例接受类固醇联合霉酚酸酯治疗的患者中有 4 例（66.66%）出现部分或完全临床缓解。未观察到严重的副作用和药物停药。
OCP 是一种影响老年人的致盲性自身免疫性疾病。虽然阳性活检结果对诊断有价值，但阴性结果不能排除诊断。主要治疗方法是全身免疫抑制剂。疾病活动度可以得到抑制，尤其是早期开始药物治疗。这些患者需要多学科方法。特别是在存在孤立性眼部表现的情况下，眼科医生应能够决定开始免疫抑制治疗，而不应延迟全身治疗。
