Risk of Cardiovascular Morbidity and Mortality in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Survivors

Abstract

Importance: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) cause diffuse epidermal detachment and necrosis. Patients who survive the initial SJS/TEN episodes are affected by various sequelae.

Objective: To investigate the risks of cardiovascular morbidity and mortality in SJS/TEN survivors.

Design, setting, and participants: This was a nationwide population-based cohort study using data from Taiwan's National Health Research Institute Database linked to the National Register of Death databases for 1998 to 2021. Survivors of SJS/TEN were identified and matched with non-SJS/TEN participants by age, sex, and Charlson Comorbidity Index. Data analysis was performed from November 2023 to June 2024.

Exposure: Cerebrovascular accidents (CVA) or ischemic heart disease (IHD) after SJS/TEN survival.

Main outcomes and measures: Cox proportional hazards models were used to estimate the hazard ratios (HRs) of CVA and IHD morbidity and mortality after SJS/TEN survival.

Results: The CVA cohort included 10 571 SJS/TEN survivors (mean [SD] age, 56.1 [18.5] years; 5358 females [50.7%] and 5213 males [49.3%]). The IHD cohort included 11 084 SJS/TEN survivors (mean [SD] age, 56.6 [18.6] years; 5561 females [50.2%] and 5523 males [49.8%]). The Cox proportional hazards model and competing risk regression model showed that compared with non-SJS/TEN participants, patients with SJS/TEN had higher risks of cardiovascular morbidity (CVA: HR, 1.65 [95% CI, 1.57-1.72] and subdistribution HR [sHR], 1.40 [95% CI, 1.33-1.46]; IHD: HR, 1.58 [95% CI, 1.51-1.65] and sHR, 1.32 [95% CI, 1.26-1.38]) and death due to cardiovascular disease (CVA: HR, 1.69; 95% CI, 1.46-1.96; IHD: HR, 1.55; 95% CI, 1.32-1.82). The increased cardiovascular mortality risks peaked at 1 year after SJS/TEN and persisted for 4 to 7 years. Older survivors and survivors admitted to an intensive care unit at SJS/TEN diagnosis had significantly higher cardiovascular mortality risk.

Conclusions and relevance: In this cohort study, SJS/TEN had a lasting association with cardiovascular function after the acute phase. This suggests a need to mitigate the elevated cardiovascular morbidity and mortality risks among survivors. Further research using databases or registries with more comprehensive clinical data are needed to validate these results.