Chronic respiratory complications in Stevens-Johnson syndrome/toxic epidermal necrolysis

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) can cause airway mucosal injury, leading to chronic respiratory complications such as bronchiolitis obliterans (BO) and small-airway disease. As SJS/TEN is rare, its chronic respiratory complications are unclear. Standardised diagnostic and management approaches remain to be established. We aimed to characterise chronic respiratory complications associated with SJS/TEN.

This retrospective single-centre observational study included 22 patients who attended a speciality ophthalmology clinic for ocular sequelae of SJS/TEN and developed respiratory symptoms after SJS/TEN. Data regarding demographics, medical history, symptoms, diagnoses, computed tomography (CT) findings, pulmonary function, and treatments were collected. CT images were evaluated for mosaic attenuation, bronchiectasis, centrilobular nodules, mucus plugs, and cysts.

The median ages of the chronic-phase patients at SJS/TEN onset and evaluation were 13.5 and 50.5 years, respectively. Overall, 77% of patients developed respiratory symptoms within 2 months of onset, whereas 95% showed CT abnormalities. Seven cases were classified as clinically diagnosed BO. Five patients showed mosaic attenuation without obstruction, which suggested small-airway disease. These cases accounted for 55% (12/22) of the symptomatic patients evaluated in this study. Inhaled therapy and long-term macrolides were administered to >50% and 27% of patients, respectively. Three patients were listed for lung transplantation.

Among patients referred for respiratory symptoms after SJS/TEN, many developed early respiratory symptoms and showed chronic-phase CT abnormalities. Assessment of new respiratory symptoms in the early phase, referral to a respiratory specialist, and long-term follow-up should be considered, even when initial imaging abnormalities are mild.