Abstract

Purpose: Over the last decade, there has been tremendous progress in the treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). To understand whether this has resulted in better ophthalmic outcomes, we aimed to study the incidence of severe ocular complications (SOCs) in the acute and chronic stage among SJS/TEN patients, major causative medications, and therapeutic effect of medical and surgical treatment.Methods: Using electronic medical records review of patients of Chang Gung Memorial Hospital Linkou Branch from 2010 to 2020, 119 patients (236 eyes) received ophthalmic consultation during the acute stage and were retrospectively studied. Sotozono's grading score systems for acute and chronic SJS/TEN were employed for accessing correlation between acute and chronic presentations, the therapeutic effect of systemic etanercept treatment, and outcome of early amniotic membrane transplantation (AMT) performed in patients with severe acute SOCs.Results: There were 46 male and 73 female patients with a mean age of 45.6 ± 22.7 years old (2–90 years), and follow-up time of 408.3 ± 351.0 (116–1,336) days. The numbers of patients with SJS, overlap syndrome, and TEN were 87, 9, and 23, respectively. In total, 109 eyes (55 patients) had acute SOCs, which comprised 46.2% of patients who underwent ophthalmic examination. Antiepileptics were the most common category of culprit drugs causing SOCs in the acute stage. At the end of follow-up, there were 14 eyes (9 patients) with chronic SOCs (5.9%), and non-steroidal anti-inflammatory drugs and cold medicine were the most common causative medications that were associated with severe chronic sequela. The correlation between Sotozono's acute and chronic grading score showed a positive relationship [Spearman's rank correlation coefficient (r) = 0.52, p < 0.001]. The average chronic grading scores in patients receiving systemic corticosteroid combined with etanercept treatment were significantly lower than those receiving corticosteroid only, Finally, the average chronic grading scores in patients receiving AMT <7 days after onset were significantly lower than those performed beyond 7 days.Conclusion: Our study implies that acute manifestation can be an indicator for chronic sequelae. Additional early etanercept treatment and early AMT showed beneficial effect in reducing chronic ocular sequela.

Highlights

  • Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a potentially life-threatening and vision-impairing immune-mediated disease with an estimated annual incidence of 1–5 per 1,000,000 individuals globally [1]

  • We aim to study the incidence of Severe ocular complications (SOCs) among SJS/TEN patients, major causative medications, the final outcome of affected patients, therapeutic effect of systemic corticosteroids combined with etanercept treatment, and beneficial effect of early amniotic membrane transplantation (AMT)

  • To investigate the effect of additional systemic etanercept on longterm ocular outcome, we focused on patients whose medical records showed treatment with intravenous corticosteroids with or without additional subcutaneous etanercept

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Summary

Introduction

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a potentially life-threatening and vision-impairing immune-mediated disease with an estimated annual incidence of 1–5 per 1,000,000 individuals globally [1]. A study from Korea reported incidences of 3.96–5.03 for SJS and 0.94–1.45 for TEN per million individuals from 2010 to 2013 [2]. An incidence rate of 5.76 SJS/TEN cases per million person-years were reported from the UK (database between 1995 and 2013) [3]. Two studies from the US reported similar incidences. The annual SJS spectrum incidence rate reported by White et al was 12.35 per million people per year (database between 2010 and 2012) [1], and Hsu et al reported that the mean estimated incidences of SJS, SJS/TEN, and TEN were 9.2, 1.6, and 1.9 per million adults per year, respectively (database between 2010 and 2012) [4]

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