史蒂文斯-约翰逊综合征的慢性眼部后遗症:病理生理学和治疗的最新进展。

IF 2.3 4区 医学 Q2 OPHTHALMOLOGY
Purvasha Narang, Sharon D'Souza
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引用次数: 0

摘要

背景:Stevens-Johnson综合征(SJS)和中毒性表皮坏死松解症(TEN)是主要由药物或感染引起的严重粘膜皮肤反应。虽然急性治疗的重点是全身稳定,但慢性眼部后遗症仍然是一个重要的问题,高达75%的受影响患者会出现视力威胁并发症。眼部表现包括干眼病、睑缘角化(LMK)和结膜瘢痕,以及更严重的并发症,如角膜缘干细胞缺乏症(LSCD)、瘢痕内翻、睑粘连、角膜穿孔和瘢痕。目的:综述SJS慢性眼部后遗症的病理生理学和治疗策略的最新进展。方法:使用PubMed数据库进行全面的文献检索,确定已发表的研究,讨论持续免疫失调、自身抗体和微生物组改变在慢性眼部炎症和瘢痕形成中的作用。最近对SJS后遗症的治疗方式也进行了评价。结论:促炎细胞因子水平升高、中性粒细胞优势和异常的角化途径已被确定为关键因素。治疗策略已经发展到包括及时的手术干预,如粘膜移植(MMG)治疗LMK,巩膜透镜治疗眼表保护,角膜缘干细胞移植治疗LSCD。引进先进的治疗方法,如局部自体血清、富血小板血浆和重组生长因子,已经增强了眼表康复。新兴的治疗方法,包括简单口腔黏膜上皮移植(SOMET)、培养口腔黏膜上皮移植(COMET)和角膜假体,为重症病例提供了希望。此外,人工智能(AI)在预后预测中的整合和靶向生物制剂的发展凸显了个性化护理的潜力。尽管取得了这些进步,但在早期诊断、获得专业护理和新疗法的高成本方面仍然存在挑战。多学科合作和意识是优化结果的必要条件。未来的方向强调需要精确医学方法、人工智能集成和全国登记,以促进研究和加强患者支持。这种全面的认识和管理方法旨在减轻SJS/TEN慢性眼部后遗症相关的长期视力障碍,从而提高生活质量。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Chronic Ocular Sequelae of Stevens-Johnson Syndrome: Recent Advances in Understanding of Patho-Physiology and Management.

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous reactions primarily triggered by drugs or infections. Although acute management focuses on systemic stabilization, chronic ocular sequelae remain a significant concern with vision-threatening complications arising in up to 75% of the affected patients. Ocular manifestations range from dry eye disease, lid margin keratinization (LMK), and conjunctival cicatrization to more severe complications like limbal stem cell deficiency (LSCD), cicatricial entropion, symblepharon, corneal perforation and scarring.

Purpose: This review aims to summarize the recent advances in understanding the pathophysiology and management strategies for chronic ocular sequelae of SJS.

Methods: A comprehensive literature search was conducted using PubMed database, identifying published studies addressing the roles of persistent immune dysregulation, autoantibodies, and microbiome alterations in perpetuating chronic ocular inflammation and scarring. The recent treatment modalities for such sequelae of SJS were also evaluated.

Conclusion: Elevated levels of pro-inflammatory cytokines, neutrophil predominance, and aberrant keratinization pathways have been identified as key contributors. Management strategies have evolved to include timely surgical interventions like mucous membrane grafting (MMG) for LMK, scleral lenses for ocular surface protection, and limbal stem cell transplantation for LSCD. The introduction of advanced therapies, such as topical autologous serum, platelet-rich plasma, and recombinant growth factors, has enhanced ocular surface rehabilitation. Emerging treatments, including simple oral mucosal epithelial transplantation (SOMET), cultivated oral mucosal epithelial transplantation (COMET), and keratoprostheses, offer hope for severe cases. Additionally, the integration of artificial intelligence (AI) in prognosis prediction and the development of targeted biologics highlight the potential for personalized care. Despite these advancements, challenges persist in early diagnosis, access to specialized care, and the high cost of novel therapies. Multidisciplinary collaboration and awareness are imperative for optimizing outcomes. Future directions emphasize the need for precision medicine approaches, AI integration, and nationwide registries to facilitate research and enhance patient support. Such comprehensive understanding and management approach aims to mitigate the long-term visual disability associated with chronic ocular sequelae of SJS/TEN, thereby improving quality of life.

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来源期刊
Seminars in Ophthalmology
Seminars in Ophthalmology OPHTHALMOLOGY-
CiteScore
3.20
自引率
0.00%
发文量
80
审稿时长
>12 weeks
期刊介绍: Seminars in Ophthalmology offers current, clinically oriented reviews on the diagnosis and treatment of ophthalmic disorders. Each issue focuses on a single topic, with a primary emphasis on appropriate surgical techniques.
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