medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/ms2023-152medsovet-7670Research ArticleТРУДНЫЙ ДИАГНОЗDIFFICULT DIAGNOSISСовременные представления о клинических вариантах и особенностях гиперсенситивного пневмонита у детейModern concepts of clinical variants and features of hypersensitivity pneumonitis in childrenhttps://orcid.org/0000-0002-8178-4630ЛевН. С.LevN. S.

Лев Наталия Сергеевна – кандидат медицинских наук, ведущий научный сотрудник отдела хронических воспалительных и аллергических болезней легких Научно-исследовательского клинического института педиатрии и детской хирургии имени академика Ю.Е. Вельтищева.

125412, Москва, ул. Талдомская, д. 2

Natalia S. Lev - Cand. Sci. (Med.), Leading Researcher of the Department of Chronic Inflammatory and Allergic Lung Diseases of the Research Clinical Institute of Pediatrics and Pediatric Surgery named after Academician Yu.E. Veltischev, Pirogov Russian National Research Medical University.

2, Taldomskaya St., Moscow, 125412

n.lev@mail.ruhttps://orcid.org/0000-0002-0740-1718МизерницкийЮ. Л.MizernitskiyYu. L.

Мизерницкий Юрий Леонидович - доктор медицинских наук, профессор, заведующий отделом хронических воспалительных и аллергических болезней легких Научно-исследовательского клинического института педиатрии и детской хирургии имени академика Ю.Е. Вельтищева.

125412, Москва, ул. Талдомская, д. 2

Yury L. Mizernitskiy - Dr. Sci. (Med.), Professor, Head of the Department of Chronic Inflammatory and Allergic Lung Diseases of the Research Clinical Institute of Pediatrics and Pediatric Surgery named after Academician Yu.E. Veltischev, Pirogov Russian National Research Medical University.

2, Taldomskaya St., Moscow, 125412

yulmiz@mail.ruРоссийский национальный исследовательский медицинский университет имени Н.И. ПироговаРоссияPirogov Russian National Research Medical UniversityRussian Federation202327072023012182191Copyright © Лев Н.С., Мизерницкий Ю.Л., 20232023Лев Н.С., Мизерницкий Ю.Л.Lev N.S., Mizernitskiy Y.L.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-sovet.pro/jour/article/view/7670

Гиперсенситивный пневмонит (J67) - самое частое интерстициальное заболевание легких у детей и подростков. Заболевание иммунологически обусловлено, может развиться при воздействии различных факторов загрязнения окружающей и домашней среды. Наиболее значимы термофильные актиномицеты, антигены животного и грибкового происхождения. В настоящее время популярна гипотеза о «двух попаданиях», когда наличие генетической предрасположенности в сочетании с воздействием провоцирующего антигена приводят к реализации болезни. Иммунологические исследования при гиперсенситивном пневмоните в основном сосредоточены на выявлении специфических IgG к «виновному» антигену. Компьютерная томография высокого разрешения является наиболее чувствительным методом визуализации гиперсенситивного пневмонита, патологические изменения выявляются более чем у 90% пациентов. По особенностям клинического течения и длительности заболевания выделяют острый (продолжительностью менее 6 мес.) и хронический (более 6 мес.) варианты болезни. Формирование фиброза легочной ткани вплоть до сотового легкого наблюдается примерно в 18% случаев. Выделяют варианты гиперсенситивного пневмонита с фиброзным и нефиброзным (воспалительным) фенотипом. Основой медикаментозной терапии до настоящего времени являются системные и ингаляционные глюкокортикостероиды, применение которых патогенетически обосновано и клинически эффективно. Перспективы терапии также связывают с использованием антифибротических препаратов: пирфенидон и нинтеданиб, которые замедляют снижение функции легких и улучшают выживаемость пациентов. В то же время ключевую роль играет исключение контакта с причинно-значимым аллергеном. Наши собственные наблюдения касаются 280 детей с гиперсенситивным пневмонитом в возрасте от 8 мес. до 16 лет, в т. ч. 70 пациентов первого года жизни. У большинства больных имелась наследственная аллергическая отягощенность (у 74%) и прослеживался причинно-следственный фактор. Прогноз, за редким исключением, был благоприятным. Основными причинами неблагоприятного исхода при гиперсенситивном пневмоните являются продолжавшийся контакт с причинно-значимым аллергеном, поздняя диагностика болезни и неадекватная терапия.

Hypersensitivity pneumonitis (HP) (J67) is the most common interstitial lung disease in children and adolescents. The disease is immunologically determined, it can be a disease when exposed to various factors that cause harmful effects on the environment. The most significant are thermophilic actinomycetes, antigens of animal and fungal origin. Currently, cases of “two cases of infection” have been identified, when a genetic predisposition has been identified in case of detection of cases of antigen results in the implementation of diseases. Immunological studies in GP are mainly focused on identifying specific IgG to the “guilty” antigen. High-resolution computed tomography is the most sensitive method of visualizing the GP, pathological changes are detected in more than 90% of patients. According to the characteristics of the clinical course and duration of the disease, acute (lasting less than 6 months) and chronic (more than 6 months) variants of the disease are distinguished. The formation of fibrosis of the lung tissue up to the honeycomb lung is observed in approximately 18% of cases. There are variants of HP: with fibrous and non-fibrous (inflammatory) phenotype. The basis of drug therapy so far is systemic and inhaled glucocorticosteroids, the use of which is pathogenetically substantiated and clinically effective. The prospects for therapy are also associated with the use of antifibrotic drugs: pirfenidone and nintedanib, which slow down the decline in lung function and improve patient survival. At the same time, the exclusion of contact with a causally significant allergen plays a key role. Our own observations concern 280 children with GP aged 8 months to 16 years, including 70 patients in the first year of life. Most patients had a hereditary allergic burden (in 74%) and a causal factor was traced. The prognosis, with rare exceptions, was favorable. The main reasons for poor outcomes in GP are continued contact with a causally significant allergen, late diagnosis of the disease, and inadequate therapy.

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The authors declare that there are no conflicts of interest present.