Matsuo Toshihiko, Tanaka Takehiro
Department of Ophthalmology, Graduate School of Interdisciplinary Science and Engineering in Health Systems, Okayama University, Okayama, JPN.
Department of Ophthalmology, Okayama University Hospital, Okayama, JPN.
Cureus. 2025 Feb 28;17(2):e79852. doi: 10.7759/cureus.79852. eCollection 2025 Feb.
Peripheral corneal infiltration, corneal ulcer, and melt are recognized complications linked to systemic immunological diseases, such as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. These manifestations, which occur in isolation, might be autoimmune keratitis but are difficult to prove underlying immunological abnormalities. This report described a patient with presumed autoimmune keratitis who repeatedly presented corneal infiltration and perforation in both eyes even after penetrating keratoplasty. The 68-year-old patient with a stable condition of keratoconjunctivitis sicca, in a 28-year follow-up, abruptly developed mild infiltrates in the corneal center of the right eye and white dense infiltrates in the peripheral and central cornea of the left eye. He was treated with topical 0.1% betamethasone eye drops and oral prednisolone tapering from 30 mg daily. The patient underwent cataract surgeries in both eyes 10 months after the onset of corneal infiltration and subsequently underwent penetrating keratoplasty in both eyes due to abrupt corneal perforation in the left eye 14 months after the onset of corneal infiltration. Six months post-keratoplasty, he experienced a recurrence of infiltrates in the corneal grafts in both eyes, leading to corneal leukoma in the left eye. The corneal graft in the right eye maintained its integrity with relatively mild opacity until approximately 3.5 years post-keratoplasty, when he abruptly developed white dense infiltration of both the corneal graft and his own peripheral cornea at the age of 73. In response to oral prednisolone tapered from 15 mg daily, the corneal infiltration in the right eye resolved but resulted in graft failure. Since he did not exhibit systemic symptoms and signs throughout the course, the repeat episodes of infiltration in both his own cornea and the corneal graft would be the manifestations of autoimmune keratitis. The entity of autoimmune keratitis in isolation would be beneficial to establish a therapeutic strategy for long-term immunosuppression in light of a risk for steroid side effects and a high rate of corneal graft failure.
周边角膜浸润、角膜溃疡和角膜溶解是与系统性免疫疾病相关的公认并发症，如抗中性粒细胞胞浆抗体（ANCA）相关血管炎。这些单独出现的表现可能是自身免疫性角膜炎，但难以证明潜在的免疫异常。本报告描述了一名疑似自身免疫性角膜炎患者，即使在穿透性角膜移植术后，双眼仍反复出现角膜浸润和穿孔。这位68岁的患者患有稳定的干燥性角结膜炎，在28年的随访中，右眼角膜中央突然出现轻度浸润，左眼周边和中央角膜出现白色致密浸润。他接受了局部0.1%倍他米松滴眼液治疗，并口服泼尼松龙，剂量从每日30毫克逐渐递减。角膜浸润发作10个月后，患者双眼接受了白内障手术，随后在角膜浸润发作14个月后，因左眼突然角膜穿孔，双眼接受了穿透性角膜移植术。角膜移植术后6个月，他双眼角膜移植片出现浸润复发，导致左眼角膜白斑。右眼的角膜移植片保持完整， opacity相对较轻，直到角膜移植术后约3.5年，73岁时，他的角膜移植片和自身周边角膜突然出现白色致密浸润。针对每日15毫克逐渐递减的口服泼尼松龙治疗，右眼的角膜浸润消退，但导致移植失败。由于他在整个病程中未出现全身症状和体征，自身角膜和角膜移植片反复出现的浸润将是自身免疫性角膜炎的表现。鉴于存在类固醇副作用风险和角膜移植失败率高，单独诊断自身免疫性角膜炎实体将有助于制定长期免疫抑制的治疗策略。
