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; = 0.002) and corneal scarring (aOR: 3.06; 95% CI, 1.15–8.14; = 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; = 0.003), progression (aOR: 11.46; 95% CI: 1.78–73.75; = 0.010), and vision loss (aOR: 3.51; 95% CI: 1.35–9.10; = 0.010). HR-EOM was associated with stage progression (aOR, 34.57; 95% CI, 6.57–181.89; <0.001) and vision loss (aOR, 8.42; 95% CI, 2.50–28.42; = 0.001). No significant differences were found between IMT regimes and relapse ( = 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. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

中文翻译：

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眼粘膜类天疱疮：就诊时危险因素对治疗结果的影响

分析目前存在的危险因素对眼粘膜类天疱疮 (OMMP) 长期免疫抑制治疗 (IMT) 结果的影响。回顾性多中心研究。 1990 年至 2022 年在杜克眼科中心、蒙特雷技术中心和巴塞罗那医院诊所就诊的 OMMP 患者。介绍的数据涉及 OMMP 患者的人口统计学、直接免疫荧光、眼部发现、眼外表现 (EOM) 部位和既往治疗情况OMMP 的临床或实验室诊断，通过多变量分析和 Kaplan-Meier 图进行分析，以确定与不良结果相关的因素。 (1)炎症控制（IMT 3个月时双眼无结膜炎症）； (2)复发（任一眼绝对控制后新发炎症）； (3) 进展（任意一只眼的愈合阶段进展≥1）； (4) 视力丧失（≥ 2 条斯内伦线）。共有 117 名患者（234 只眼），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（四分位数范围：16-66 个月；范围：3-265 个月），被纳入。 57% 的高危 EOM (HR-EOM) 患者 (67/117) 实现了炎症控制，包括食管、鼻咽和/或生殖器受累（调整后比值比 [aOR]：12.51；95% 置信区间[CI]：2.61–59.99；= 0.002）和角膜疤痕（aOR：3.06；95% CI，1.15–8.14；= 0.025），是持续性炎症的重要危险因素。疾病复发、进展和视力丧失的发生率分别为 20% (23/117)、12% (14/117) 和 27% (32/117) 的患者。基线角膜疤痕是复发（调整后风险比：4.14；95% CI：1.61-10.62；= 0.003）、进展（aOR：11.46；95% CI：1.78-73.75；= 0.010）和视力丧失的危险因素（ aOR：3.51；95% CI：1.35-9.10；= 0.010）。 HR-EOM 与分期进展（aOR，34.57；95% CI，6.57-181.89；<0.001）和视力丧失（aOR，8.42；95% CI，2.50-28.42；= 0.001）相关。 IMT 方案和复发之间没有发现显着差异 (= 0.169)。伴有 HR-EOM 和角膜疤痕的眼粘膜类天疱疮会增加分期进展和视力丧失的风险。基线时的角膜疤痕和严重炎症与复发风险增加相关。需要结合 HR-EOM 和角膜受累的疾病进展分期系统来更好地预测 OMMP 的视觉结果。专有或商业披露可在本文末尾的脚注和披露中找到。