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Автозапальні захворювання. Частина 3: NLRP та NLRC інфламасомопатії. Біологічна терапія інфламасомопатій

Автозапальні захворювання. Частина 3: NLRP та NLRC інфламасомопатії. Біологічна терапія інфламасомопатій

Авторы: Шварацька О.В., Бордій Т.А., Калічевська М.В., Клименко О.В., Таран О.М., Клімова О.В., Віленський Я.В., Мавропуло Т.К. Дніпровський державний медичний університет, м. Дніпро, Україна

Рубрики: Педиатрия/Неонатология

Разделы: Справочник специалиста

Версия для печати


Резюме

Інфламасомопатії та інші синдроми посилення сигналу інтерлейкіну (IL)-1 є значущим кластером системних автозапальних захворювань (САЗЗ) — моногенних станів, які характеризуються епізодичним спонтанним полісистемним запаленням, що виникає переважно через невідповідну активацію антиген-незалежних запальних механізмів без участі автоантитіл, тобто опосередковується механізмами вродженого імунітету. Автозапалення наразі розглядається як окрема категорія імунної дисфункції поряд з автоімунітетом/алергією та імунодефіцитом. Цей науковий огляд є третім у серії публікацій, об’єднаних загальною метою підвищення обізнаності лікарів про діагностику та менеджмент САЗЗ у дітей. В огляді висвітлені імунобіологічні особливості інфламасом, які містять білки з нуклеотидзв’язувальним доменом, що містять повтори з високим вмістом лейцину (NLR), та надана клініко-патогенетична характеристика генетично визначених NLRP3-, NLRP12-, NLRP1- та NLRC4-асоційованих інфламасомопатій. Окрім цього, в роботі обговорено потенціал таргетної біологічної терапії інфламасомопатій, що ґрунтується на молекулярному імунопатогенезі захворювання і яка наразі вважається ключовим напрямом лікування САЗЗ. Також нами було узагальнено сучасний досвід використання специфічних біологічних препаратів у терапії окремих інфламасомопатій, а саме засобів, спрямованих на блокаду ефектів IL-1, IL-6, IL-17, IL-12/23, інтерферону-гамма, фактора некрозу пухлини тощо. Пошук інформації за останні 10 років здійснювався по базах даних Web of Science, Scopus, PubMed Central®, Google Scholar за ключовими словами: autoinflammatory diseases, autoinflammation, inflammasomopathies, NLRP, NLRC, biologic therapies.

Inflammasomopathies and other enhanced interleukin (IL)-1 signaling syndromes represent a significant cluster of systemic autoinflammatory diseases (SAIDs) which are monogenic disorders characterized by episodic spontaneous multisystemic inflammation mediated by innate immunity and resulting mainly from inappropriate activation of antigen-independent inflammatory mechanisms without production of autoantibodies. Autoinflammation is currently considered a distinct category of immune dysfunction along with autoimmunity/allergy and immunodeficiency. This scientific review is the third in a series of publications aimed at raising awareness among healthcare professionals regarding the diagnosis and management of SAIDs in children. The review highlights the immunobiological features of inflammasomes containing nucleotide-binding domain, leucine-rich repeat-containing proteins (NLRs). It also presents the clinical and pathogenetic features of the currently genetically defined NLRP3-, NLRP12-, NLRP1- and NLRC4-associated inflammasomopathies. The paper also discusses the potential of biologic-targeted therapies for inflammasomopathies which are now considered a key trend in the treatment of SAIDs and based on the molecular immunopathogenesis of the disease. Furthermore, we summarized the current experience of using available biological agents in the treatment of specific inflammasomopathies, namely agents blocking the effects of IL-1, IL-6, IL-17, IL-12/23, interferon-gamma, and tumor necrosis factor. Data were collected through a focused search over the Web of Science, Scopus, PubMed Central®, Google Scholar databases for the past 10 years using the keywords “autoinflammatory diseases”, “autoinflammation”, “inflammasomopathies”, “NLRP”, “NLRC”, “biologic therapies”.


Ключевые слова

автозапальні захворювання; інфламасомопатії; інтерлейкін 1; біологічні препарати; терапія; огляд

autoinflammatory diseases; inflammasomopathies; interleukin 1; biologics; treatment; review

doi: http://dx.doi.org/10.22141/2224-0551.20.2.2025.1808

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