Department of Ophthalmology, Eastern Virginia Medical School, Norfolk, VA.
Department of Pediatrics (Nephrology), Children's Hospital of The King's Daughters, Norfolk, VA; and.
Cornea. 2023 Jun 1;42(6):747-750. doi: 10.1097/ICO.0000000000003229. Epub 2023 Jan 10.
The aim of this study was to describe the use of intravenous immunoglobulin (IVIG) in the management of a 20-year-old woman with autoimmune polyglandular syndrome-associated keratopathy who developed acute transplant rejection after keratolimbal allograft (KLAL) surgery.
Nine weeks after KLAL surgery, a 20-year-old woman with autoimmune polyglandular syndrome-related limbal stem cell deficiency presented with graft injection, hemorrhage, and an epithelial rejection line. This was concerning for acute rejection in the setting of triple-agent systemic immunosuppression (albeit nonadherence at times). There was initial reversal of the rejection process with a sub-Tenon's injection of triamcinolone, frequent topical corticosteroids, increase in oral prednisone, and optimization of systemic immunosuppression medications; however, recurrence of the epithelial rejection line and symptoms were noted whenever the prednisone dose was tapered. This was accompanied by ocular surface decompensation (late staining, neovascularization, and persistent epithelial defects). She was found to have weakly positive HLA Class 1 antibodies. The patient was treated with a pulsed corticosteroid infusion and 2 monthly IVIG infusions. This led to resolution of the acute rejection. However, there was a subsequent rejection episode 4 months later after tapering the prednisone. Monthly IVIG for 6 more months led to final resolution with successful prednisone tapering and no further rejection.
Treatment with prolonged IVIG showed better improvement in a case of acute rejection refractory to traditional treatments, especially in the setting of HLA antibodies. The case demonstrates that close follow-up with a corneal specialist and collaboration with a transplant specialist is important to monitor for postoperative KLAL rejection.
本研究旨在描述静脉注射免疫球蛋白（IVIG）在治疗一名 20 岁女性自身免疫性多腺体综合征相关角结膜炎患者中的应用。该患者在角膜缘异体干细胞移植（KLAL）手术后发生急性移植排斥反应。
KLAL 手术后 9 周，一名 20 岁女性因自身免疫性多腺体综合征相关角膜缘干细胞缺乏症出现移植物注射、出血和上皮排斥线，这在三联药物全身免疫抑制（尽管有时不遵守）的情况下提示急性排斥反应。尽管有时不遵守）的情况下提示急性排斥反应。起初，通过经Tenon 下注射曲安奈德、频繁局部皮质类固醇、增加口服泼尼松龙以及优化全身免疫抑制药物来逆转排斥过程，但每当泼尼松龙剂量减少时，上皮排斥线和症状都会再次出现。这伴随着眼表失代偿（晚期染色、新生血管形成和持续的上皮缺损）。发现患者 HLA 类 1 抗体呈弱阳性。该患者接受了脉冲皮质类固醇输注和 2 次每月 IVIG 输注治疗。这导致急性排斥反应得到缓解。然而，在随后的 4 个月，在减少泼尼松龙剂量后，又发生了一次排斥发作。6 个月内每月接受 IVIG 治疗导致最终缓解，成功减少了泼尼松龙的剂量，且无进一步排斥反应。
在对传统治疗方法产生抗药性的急性排斥反应病例中，长期使用 IVIG 治疗显示出更好的改善效果，尤其是在 HLA 抗体的情况下。该病例表明，密切随访角膜专家并与移植专家合作对于监测 KLAL 术后排斥反应非常重要。
