Annals of the Russian academy of medical sciencesAnnals of the Russian academy of medical sciencesВестник Российской академии медицинских наук0869-60472414-3545"Paediatrician" Publishers LLC105110.15690/vramn1051DERMATOLOGY and VENEROLOGY: current issuesАКТУАЛЬНЫЕ ВОПРОСЫ ДЕРМАТОВЕНЕРОЛОГИИResearch ArticleEvaluation of the Effectiveness of Therapy for Stevens−Johnson Syndrome and Toxic Epidermal Necrolysis: A ReviewСравнительная оценка эффективности различных методов терапии синдрома Стивенса−Джонсона и токсического эпидермального некролиза на основании данных литературыhttps://orcid.org/0000-0002-4365-30903261-3520LepekhovaAnfisa A.ЛепеховаАнфиса Александровна
Russian Federation

MD, PhD, Associate professor

кандидат медицинских наук, доцент кафедры кожных и венерических болезней им. В.А. Рахманова

anfisa.lepehova@yandex.ruhttps://orcid.org/0000-0003-0751-00737765-7631AllenovaAnastasiya S.АлленоваАнастасия Сергеевна
Russian Federation

MD, PhD, assistant researcher

младший научный сотрудник НИО Иммунозависимых дерматозов Научно-технологического парка биомедицины, кандидат медицинских наук

erika-mma@yandex.ruhttps://orcid.org/0000-0003-2482-17542500-7989OlisovaOlga Yu.ОлисоваОльга Юрьевна
Russian Federation

MD, PhD, professor

доктор медицинских наук, кафедра кожных и венерических болезней им. В.А. Рахманова, заведующая кафедрой, профессор

olisovaolga@mail.ruhttps://orcid.org/0000-0002-5800-48008013-3256TeplyukNataliya P.ТеплюкНаталия Павловна
Russian Federation

MD, PhD, professor

доктор медицинских наук, профессор кафедры кожных и венерических болезней им. В.А. Рахманова

Teplyukn@gmail.ruhttps://orcid.org/0000-0001-5846-87128964-2703KanareikinaElizaveta V.КанарейкинаЕлизавета Вячеславовна
Russian Federation

student

студентка 6-го курса, 62 группа ЛФ

liza.kanareikina@mail.ruI.M. Sechenov First Moscow State Medical University (Sechenov University)Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет)270720197431571661010201816062019Copyright ©; 2019, "Paediatrician" Publishers LLCCopyright ©; 2019, Издательство "Педиатръ"2019"Paediatrician" Publishers LLCИздательство "Педиатръ"https://vestnikramn.spr-journal.ru/jour/article/view/1051

39 original articles were analyzed. 8 of them were excluded due to the small sample of patients. The effectiveness of the intravenous Ig, systemic glucocorticosteroids, cyclosporin A, biological agents (etranecept, infliximab, thalidomide) on the basis of the SCORTEN scale and the number of lethal outcomes was evaluated. Information was searched for the following databases: PubMed, ScienceDirect, Wiley Online Library, Google Scholar, Cochrane Library. The systemic glucocorticosteroid (GCs) pulse therapy using only in the phase of disease progression was the most appropriate. Some authors showed a high risk of sepsis development in patients treated with GCs. In patients who received high doses of IVIg (≥2 g/ kg), mortality was 2.5 times lower compared to the lower one. The number of lethal outcomes in the Cyclosporin A (3 mg/kg/day) group was 3.3 times lower. A high mortality rate was observed in patients receiving thalidomide. The effectiveness of a particular method of therapy, as well as the prognosis of the disease, largely depends on the process severity, the presence and type of concomitant pathology (for example, severe course and negative outcome in patients with cancer). There is no a single point of view regarding the therapy of SSJ and TEN. Thus, a large multicenter randomized studies are crucial.

Авторами выполнен анализ 39 оригинальных исследований, касающихся изучения эффективности терапии токсического эпидермального некролиза (ТЭН) и синдрома Стивенса−Джонсона (ССД), 8 из которых были исключены ввиду малой выборки больных, а также применения комбинированной терапии. В работе оценена эффективность применения внутривенного иммуноглобулина (IVIg), системных глюкокортикостероидов, циклоспорина А, биологических препаратов (этарнерцепт, инфликсимаб), талидомида и плазмафереза в лечении ССД, ТЭН, ССД/ТЭН на основании шкалы SCORTEN и уровня смертности. Наиболее целесообразным в лечении ТЭН оказалось применение пульс-терапии системными глюкокортикостероидами (1000 мг в течение 3 дней) только в фазе прогрессирования заболевания. Другие исследователи указывали на высокий риск развития сепсиса при применении системных глюкокортикостероидов. У больных, получавших высокие дозы IVIg (≥2 г/кг), смертность была в 2,5 раза ниже по сравнению с меньшими дозами IVIg. Количество летальных исходов в группе циклоспорина A (3 мг/кг в сутки) было в 3,3 раза ниже. Плазмаферез может применяться при неэффективности других препаратов или в качестве дополнительной терапии. Высокий уровень смертности наблюдался у больных, получавших талидомид. Таким образом, эффективность того или иного метода терапии, а также прогноз заболевания в большей степени зависят от тяжести процесса, наличия и вида сопутствующей патологии (например, тяжелое течение и негативный исход у онкологических пациентов), что говорит о необходимости проведения крупных многоцентровых рандомизированных клинических и экспериментальных исследований в будущем.

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