Department of Ophthalmology, Foster Center for Ocular Immunology at Duke Eye Center, Duke University School of Medicine, Durham, North Carolina; Tecnologico de Monterrey, School of Medicine and Health Sciences, Institute of Ophthalmology and Visual Sciences, Ocular Immunology and Uveitis Service, Monterrey, Mexico.
Tecnologico de Monterrey, School of Medicine and Health Sciences, Institute of Ophthalmology and Visual Sciences, Ocular Immunology and Uveitis Service, Monterrey, Mexico.
Ophthalmology. 2024 Sep;131(9):1064-1075. doi: 10.1016/j.ophtha.2024.02.028. Epub 2024 Mar 6.
Analyze the influence of risk factors at presentation in the long-term immunosuppressive therapy (IMT) outcomes of ocular mucous membrane pemphigoid (OMMP).
Retrospective multicenter study.
Patients with OMMP seen at the Duke Eye Center, Tecnologico de Monterrey, and Hospital Clinic of Barcelona from 1990 to 2022.
Data at presentation on demographics, direct immunofluorescence, ocular findings, sites of extraocular manifestations (EOMs), and previous treatments in patients with a clinical or laboratory diagnosis of OMMP, were analyzed with multivariable analysis and Kaplan-Meier plots to identify factors associated with adverse outcomes.
(1) Inflammatory control (no conjunctival inflammation in both eyes at 3 months on IMT); (2) relapse (new-onset inflammation after absolute control in either eye); (3) progression (≥ 1 cicatrizing stage progression in either eye); and (4) vision loss (≥ 2 Snellen lines).
A total of 117 patients (234 eyes), 61% (71/117) of whom were women, with a mean age of 66.6 (SD: 12.4) years (range: 37-97 years) and median follow-up of 34 months (interquartile range: 16-66 months; range: 3-265 months), were enrolled. Inflammatory control was achieved in 57% of patients (67/117), with high-risk EOM (HR-EOM), including esophageal, nasopharyngeal, and/or genital involvement (adjusted odds ratio [aOR]: 12.51; 95% confidence interval [CI]: 2.61-59.99; P = 0.002) and corneal scarring (aOR: 3.06; 95% CI, 1.15-8.14; P = 0.025), as significant risk factors for persistent inflammation. Disease relapse, progression, and vision loss occurred in 20% of patients (23/117), 12% of patients (14/117), and 27% of patients (32/117), respectively. Baseline corneal scarring was a risk factor for relapse (adjusted hazard ratio: 4.14; 95% CI: 1.61-10.62; P = 0.003), progression (aOR: 11.46; 95% CI: 1.78-73.75; P = 0.010), and vision loss (aOR: 3.51; 95% CI: 1.35-9.10; P = 0.010). HR-EOM was associated with stage progression (aOR, 34.57; 95% CI, 6.57-181.89; P<0.001) and vision loss (aOR, 8.42; 95% CI, 2.50-28.42; P = 0.001). No significant differences were found between IMT regimes and relapse (P = 0.169).
Ocular mucous membrane pemphigoid presenting with HR-EOMs and corneal scarring has an increased risk of stage progression and vision loss. Corneal scarring and severe inflammation at baseline were associated with an increased risk of relapse. A disease progression staging system incorporating both the HR-EOMs and corneal involvement is required to predict the visual outcome of OMMP better.
FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
分析在长期免疫抑制治疗（IMT）中，眼部黏膜炎型天疱疮（OMMP）患者的表现相关风险因素对其治疗结局的影响。
回顾性多中心研究。
1990 年至 2022 年期间在杜克眼科中心、蒙特雷技术大学和巴塞罗那临床医院就诊的 OMMP 患者。
对具有 OMMP 临床或实验室诊断的患者，分析其就诊时的人口统计学资料、直接免疫荧光检查、眼部表现、眼外表现部位（EOM）以及既往治疗情况等数据。采用多变量分析和 Kaplan-Meier 图来识别与不良结局相关的因素。
（1）炎症控制（在 IMT 治疗的第 3 个月时双眼均无结膜炎症）；（2）复发（在任何一只眼的炎症完全控制后出现新的炎症）；（3）进展（任何一只眼的至少 1 个瘢痕性阶段进展）；（4）视力丧失（至少 2 行 Snellen 视力表）。
共纳入 117 例（234 只眼）患者，其中 61%（71/117）为女性，平均年龄为 66.6（SD：12.4）岁（范围：37-97 岁），中位随访时间为 34 个月（四分位距：16-66 个月；范围：3-265 个月）。57%（67/117）的患者达到了炎症控制，存在高风险 EOM（HR-EOM），包括食管、鼻咽和/或生殖器受累（校正优势比 [aOR]：12.51；95%置信区间 [CI]：2.61-59.99；P=0.002）和角膜瘢痕（aOR：3.06；95%CI，1.15-8.14；P=0.025）是持续炎症的显著危险因素。20%（23/117）的患者出现疾病复发，12%（14/117）的患者出现疾病进展，27%（32/117）的患者出现视力丧失。基线时的角膜瘢痕是复发（校正风险比：4.14；95%CI：1.61-10.62；P=0.003）、进展（aOR：11.46；95%CI：1.78-73.75；P=0.010）和视力丧失（aOR：3.51；95%CI：1.35-9.10；P=0.010）的危险因素。HR-EOM 与阶段进展（aOR，34.57；95%CI，6.57-181.89；P<0.001）和视力丧失（aOR，8.42；95%CI，2.50-28.42；P=0.001）相关。不同 IMT 方案与复发之间无显著差异（P=0.169）。
患有 HR-EOM 和角膜瘢痕的眼部黏膜炎型天疱疮患者发生疾病进展和视力丧失的风险增加。基线时的角膜瘢痕和严重炎症与复发风险增加相关。需要建立一个包含 HR-EOM 和角膜受累的疾病进展分期系统，以更好地预测 OMMP 的视力结局。
本文末尾的脚注和披露中可能包含专有或商业披露信息。
