Efficacy and safety of intravenous immunoglobulin treatment of ocular steven-johnson syndrome/toxic epidermal necrolysis in young patients

Abstract

Background: there is minimal evidence regarding treatment with intravenous immunoglobulins (IVIG) and its effects on ocular outcomes in Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN).

Aim: to evaluate the efficacy and safety of IVIG in the treatment of ocular SJS/TEN.

Methods: a retrospective case series at University Hospital Southampton between 2016-2024. Inclusion criteria, systemic and ocular symptoms of SJS/TEN, skin biopsy, treatment with IVIG, systemic and intensive topical steroid treatment.

Ocular findings at presentation and at six months post-treatment were investigated. The acute ocular severity was graded using the Gregory grading score, while the chronic ocular complications the Sotozono system.

Results: five patients included, four between 5-6, one 23 years old. The mean time to initiate IVIG from the onset of symptoms was 3 days (range 1-5). Four received 2 g/kg and one 1 g/kg, in two divided doses over 2 days. All survived, and no serious side effects were observed. Three patients presented with very severe ocular findings, including lid involvement, hyperaemia, symblepharon, and epithelial defects. One patient exhibited severe lid involvement, hyperaemia, and epithelial defect. Another experienced mild acute ocular involvement. Three patients underwent amniotic membrane grafting. One patient with high SCORTEN and 95% BSA detached skin, developed chronic ocular complications of hyperaemia, corneal dryness/conjunctivalisation, symblepharon, bulbar keratinisation. Two patients experienced mild corneal dryness, while all four other showed resolution of inflammatory changes, and no complications.

Conclusion: this case series provides evidence for the ocular management of patients with SJS/TEN and supports treatment with a combination of systemic and intensive topical steroids, amniotic membrane, and intravenous immunoglobulins.

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ORCID for Parwez Hossain: orcid.org/0000-0002-3131-2395

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