Russian Journal of AllergyRussian Journal of AllergyРоссийский Аллергологический Журнал1810-88302686-682XPublishing House ABV Press1704310.36691/RJA17043Case reportsКлинические случаиResearch ArticleBronchiolitis 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 comparisonsОблитерирующий бронхиолит — летальное осложнение синдрома Стивенса–Джонсона/токсического эпидермального некролиза у подростка с эпилепсией, получавшего терапию ламотриджином и нестероидными противовоспалительными препаратами: клинико-морфологические сопоставленияhttps://orcid.org/0000-0002-4961-384X5249-5760OvsyannikovDmitriy Yu.ОвсянниковДмитрий Юрьевич
Russian Federation

MD, Dr. Sci. (Medicine), Professor

д-р мед. наук, профессор

mdovsyannikov@yahoo.comhttps://orcid.org/0000-0003-2375-46253077-6589BykovIlya A.БыковИлья Андреевич
Russian Federation

MD

svgkofein@yandex.ruhttps://orcid.org/0000-0001-6232-544X7062-6008GitinovShamil A.ГитиновШамиль Абдулвахидович
Russian Federation

MD

dr.gitinov@mail.ruhttps://orcid.org/0000-0003-1057-05363852-6705AsatryanSuzanna P.АсатрянСюзанна Павловна
Russian Federation

MD

syzanna_pavlovna@mail.ruhttps://orcid.org/0000-0003-2158-6672BrunovaOlga Yu.БруноваОльга Юрьевна
Russian Federation

MD

oubrunova@yandex.ruhttps://orcid.org/0009-0009-6241-91422902-2501ValievaSaniya I.ВалиеваСания Ириковна
Russian Federation

MD, Dr. Sci. (Medicine), Professor

д-р мед. наук, профессор

valieva.sania@yandex.ruhttps://orcid.org/0000-0001-8272-36488944-9664GorevValeriy V.ГоревВалерий Викторович
Russian Federation

MD, Cand. Sci. (Medicine)

канд. мед. наук

mdgkb@zdrav.mos.ruhttps://orcid.org/0000-0003-4019-31889402-2169DavydovIgor S.ДавыдовИгорь Сергеевич
Russian Federation

MD

i@davidov41.ruhttps://orcid.org/0000-0002-0352-25639924-0270DeevaEvgeniya V.ДееваЕвгения Викторовна
Russian Federation

MD, Cand. Sci. (Medicine)

канд. мед. наук

evgenia.v.deeva@gmail.comhttps://orcid.org/0000-0002-4514-84694363-1578ZiminSergey B.ЗиминСергей Борисович
Russian Federation

MD

zimin-sb@rambler.ruhttps://orcid.org/0000-0001-6012-250X4748-1308KesselAleksandr E.КессельАлександр Евгеньевич
Russian Federation

MD

kesselae@yandex.ruhttps://orcid.org/0000-0003-1174-07369251-5267MalyshevOleg G.МалышевОлег Геннадьевич
Russian Federation

MD

omalyshev03@vk.comhttps://orcid.org/0000-0001-5039-84739722-7961PampuraAleksandr N.ПампураАлександр Николаевич
Russian Federation

MD, Dr. Sci. (Medicine), Professor

д-р мед. наук, профессор

apampura1@mail.ruhttps://orcid.org/0000-0002-0348-19259938-7840TalalaevAleksandr G.ТалалаевАлександр Гаврилович
Russian Federation

MD, Dr. Sci. (Medicine), Professor

д-р мед. наук, профессор

talalaev@mail.ruhttps://orcid.org/0000-0003-4994-71936302-3406TigayZhanna G.ТигайЖанна Геннадьевна
Russian Federation

MD, Dr. Sci. (Medicine), Professor

д-р мед. наук, профессор

shekz@mail.ruPeoples’ Friendship University of RussiaРоссийский университет дружбы народов имени Патриса ЛумумбыMorozov Children’s City Clinical HospitalМорозовская детская городская клиническая больницаRussian Medical Academy of Continuous Professional EducationРоссийская медицинская академия непрерывного профессионального образованияThe Russian National Research Medical University named after N.I. PirogovРоссийский национальный исследовательский медицинский университет имени Н.И. Пирогова09092025101020252233353492607202505092025Copyright ©; 2025, ABV-pressCopyright ©; 2025, ИД "АБВ-пресс"2025ABV-pressИД "АБВ-пресс"https://rusalljournal.ru/raj/article/view/17043

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.

Облитерирующий бронхиолит — редкое тяжелое осложнение синдрома Стивенса–Джонсона и токсического эпидермального некролиза.

В статье представлены результаты наблюдения летального гистологически подтвержденного облитерирующего бронхиолита у 16-летней пациентки, возникшего как отсроченное осложнение синдрома Стивенса–Джонсона после применения ламотриджина и нестероидных противовоспалительных препаратов. Диагноз облитерирующего бронхиолита был установлен на основании развития тяжелого бронхообструктивного синдрома, подтвержденного по данным исследования функции внешнего дыхания, хронической респираторной недостаточности через 2 мес после начала синдрома Стивенса–Джонсона, характерных признаков по данным компьютерной томографии (очаги мозаичной перфузии, бронхоэктазы). Терапия облитерирующего бронхиолита, кроме обычно применяемых препаратов, включала ингибитор янус-киназ тофацитиниб.

Для обсуждения результатов клинического наблюдения проведен систематический обзор мировой литературы за 45 лет. Из 187 публикаций отобрано 43 описания случая облитерирующего бронхиолита после синдрома Стивенса–Джонсона/токсического эпидермального некролиза с анализом этиологии, сроков дебюта, спирометрических и рентгенологических признаков, особенностей терапии и течения. По результатам проведенного анализа основными триггерами синдрома Стивенса–Джонсона/токсического эпидермального некролиза послужили антибиотики (50 %) и нестероидные противовоспалительные препараты (40 %), инфекция, вызванная Mycoplasma pneumoniae (12 %), и реже антиконвульсанты. Средний возраст детей с облитерирующим бронхиолитом составил 7 лет, взрослых — 28 лет. Манифестация облитерирующего бронхиолита в 50 % случаев происходила через 1–3 мес после начала синдрома Стивенса–Джонсона/токсического эпидермального некролиза. У большинства (35 %) пациентов облитерирующий бронхиолит манифестировал тяжелым бронхообструктивным синдромом, характерные признаки по данным компьютерной томографии включали мозаичную перфузию (75 %), бронхоэктазы (49 %). Основу терапии облитерирующего бронхиолита составляли системные (77 %) и ингаляционные (35 %) глюкокортикостероиды, бронходилататоры (63 %), макролидные антибиотики (26 %). Летальность в анализируемых случаях достигала 30 %, полное выздоровление наблюдалось лишь в 33 %, а у 35 % пациентов сохранялся стойкий бронхообструктивный синдром.

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