The natural history of Stevens Johnson syndrome: patterns of chronic ocular disease and the role of systemic immunosuppressive therapy

Abstract

Objective: To characterize patterns of chronic ocular disease in patients with Stevens-Johnson syndrome (SJS) and its variant toxic epidermal necrolysis (TEN), and to describe their response to treatment.

Methods: Retrospective case series. A review of hospital records of 30 patients (60 eyes) with ocular manifestations of SJS or TEN was carried out. The principal outcome measure was to identify and classify the patterns of chronic ocular disease in SJS and TEN. The secondary outcome measure was the response to treatment.

Results: Patterns of chronic ocular disease observed after the acute episode included: mild/moderate SJS, severe SJS, ocular surface failure (SJS-OSF), recurrent episodic inflammation (SJS-RI), scleritis (SJS-S) and progressive conjunctival cicatrisation resembling mucous membrane pemphigoid (SJS-MMP). The median follow-up was 5 years (range 0-29). 19 patients (29 eyes (48%)) developed SJS-OSF, SJS-RI, SJS-S or SJS-MMP during follow-up. SJS-OSF was present in 12 patients (18 eyes (30%)). In 5 patients (eight eyes) this developed 1 year after the acute illness, without any further inflammatory episodes; it was associated with SJS-RI in 1 patient (2 eyes), with SJS-RI and SJS-S in 1 patient (1 eye), with SJS-S in 1 patient (1 eye) and with SJS-MMP in 4 patients (6 eyes). Episodes of SJS-RI occurred in 4 patients (7 eyes (12%)). The median time from acute disease to the first episode of SJS-RI was 8.5 years (range 5-63). SJS-S developed in 2 patients (4 eyes (7%)), of which 2 eyes subsequently developed SJS-OSF. SJS-MMP developed in 5 patients (10 eyes (16.6%)). The median duration from the acute stage to the diagnosis of SJS-MMP was 2 years (range 1-14). Immunosuppressive therapy successfully controlled inflammation in 10/10 patients with SJS-MMP, SJS-RI or SJS-S.

Conclusion: Ocular disease in SJS/TEN is not limited solely to the sequelae of the acute phase illness. Patients and physicians need to know that ocular disease progression, due to surface failure and/or acute inflammatory conditions, may occur at variable periods following the acute disease episode. Recognition of this, and prompt access to specialist services, may optimise management of these uncommon patterns of disease in SJS.