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 (OB) is a rare severe complication of Stevens—Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). The article presents an observation of a fatal histologically confirmed OB in a 16-year-old patient who developed as a delayed complication of SJS after the use of lamotrigine and nonsteroidal anti-inflammatory drugs (NSAIDs). The diagnosis of OB was established based on the development of severe bronchoobstructive syndrome (BOS), confirmed by a study of the function of external respiration, chronic respiratory failure 2 months after SSD, characteristic CT signs (foci of mosaic perfusion, bronchiectasis). OB 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-SJS/TEN 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 SSD/TEN were antibiotics (50%) and NSAIDs (40%), infection caused by Mycoplasma pneumoniae (12%), and less often anticonvulsants. The average age of children with OB was 7 years, and the average age of adults was 28 years. In 50% of cases, the manifestation of OB occurred 1-3 months after the start of SSD/TEN. Most patients (35%) had severe BOS, and characteristic CT signs included mosaic perfusion (75%) and bronchiectasis (49%). Systemic (77%) and inhaled (35%) glucocorticosteroids, bronchodilators (63%), and macrolide antibiotics (26%) formed the basis of OB therapy. Mortality in the analyzed cases reached 30%, complete recovery was observed in only 33%, and 35% of patients retained persistent BOS.