Bronchiolitis obliterans is a fatal complication of Stevens–Johnson syndrome/toxic epidermal necrolysis in an adolescent with epilepsy treated with lamotrigine and nonsteroidal anti-inflammatory drugs: clinical and morphological comparisons

Bronchiolitis obliterans is a rare severe complication of Stevens–Johnson syndrome and toxic epidermal necrolysis. The article presents an observation of a fatal histologically confirmed bronchiolitis obliterans in a 16-year-old patient developed as a delayed complication of Stevens–Johnson syndrome after the use of lamotrigine and nonsteroidal anti-inflammatory drugs. The diagnosis of bronchiolitis obliterans was established based on the development of severe bronchoobstructive syndrome, confirmed by a study of the external respiratory function, chronic respiratory failure 2 months after Stevens–Johnson syndrome, characteristic computed tomography signs (foci of mosaic perfusion, bronchiectasis). Bronchiolitis obliterans therapy, in addition to commonly used drugs, included the janus kinase inhibitor tofacitinib. To discuss clinical observation, a systematic review of the world literature over 45 years was conducted. 43 cases of post-Stevens–Johnson syndrome/toxic epidermal necrolysis were selected from 187 publications with an analysis of the etiology, timing of onset, spirometric and radiological signs, features of therapy and course. According to the analysis, the main triggers of Stevens–Johnson syndrome/toxic epidermal necrolysis were antibiotics (50 %) and nonsteroidal anti-inflammatory drugs (40 %), infection caused by Mycoplasma pneumoniae (12 %), and less often anticonvulsants. The average age of children with bronchiolitis obliterans was 7 years, and the average age of adults was 28 years. In 50 % of cases, the manifestation of bronchiolitis obliterans occurred 1–3 months after the start of Stevens–Johnson syndrome/toxic epidermal necrolysis. Most patients (35 %) had severe bronchoobstructive syndrome, and characteristic computed tomography signs included mosaic perfusion (75 %) and bronchiectasis (49 %). Systemic (77 %) and inhaled (35 %) glucocorticosteroids, bronchodilators (63 %), and macrolide antibiotics (26 %) formed the basis of bronchiolitis obliterans therapy. Mortality in the analyzed cases reached 30 %, complete recovery was observed in only 33 %, and 35 % of patients retained persistent bronchoobstructive syndrome.