The ClinicianThe ClinicianКлиницист1818-83382412-8775Publishing House ABV Press51310.17650/1818-8338-2022-16-4-K658REVIEWОБЗОРBiomarkers of interstitial lung disease in systemic scleroderma and their significanceБиомаркеры интерстициального заболевания легких при системной склеродермии и их значениеhttps://orcid.org/0000-0001-5357-804XKhorolskyD. V.ХорольскийД. В.
Russian Federation

Dmitry Vyacheslavovich Khorolsky, Acad. A.I. Nesterov of Faculty Therapy

1 Ostrovityanova St., Moscow 117997

Дмитрий Вячеславович Хорольский, Кафедра факультетской терапии им. акад. А. И. Нестерова

117997 Москва, ул. Островитянова, 1

pchelkins86@yandex.ruhttps://orcid.org/0000-0002-7410-9784KlimenkoA. A.КлименкоА. А.
Russian Federation

Acad. A.I. Nesterov of Faculty Therapy

1 Ostrovityanova St., Moscow 117997

Кафедра факультетской терапии им. акад. А. И. Нестерова

117997 Москва, ул. Островитянова, 1

https://orcid.org/0000-0001-9152-3234KondrashovA. A.КондрашовА. А.
Russian Federation

Acad. A.I. Nesterov of Faculty Therapy

1 Ostrovityanova St., Moscow 117997

Кафедра факультетской терапии им. акад. А. И. Нестерова

117997 Москва, ул. Островитянова, 1

https://orcid.org/0000-0003-4669-1006ShostakN. A.ШостакН. А.
Russian Federation

Acad. A.I. Nesterov of Faculty Therapy

1 Ostrovityanova St., Moscow 117997

Кафедра факультетской терапии им. акад. А. И. Нестерова

117997 Москва, ул. Островитянова, 1

https://orcid.org/0000-0001-6890-8777DemidovaN. A.ДемидоваН. А.
Russian Federation

Acad. A.I. Nesterov of Faculty Therapy

1 Ostrovityanova St., Moscow 117997

Кафедра факультетской терапии им. акад. А. И. Нестерова

117997 Москва, ул. Островитянова, 1

N. I. Pirogov Russian National Research Medical University, Ministry of Health of RussiaФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н. И. Пирогова» Минздрава России1510202216410180902202309022023Copyright ©; 2022, Khorolsky D.V., Klimenko A.A., Kondrashov A.A., Shostak N.A., Demidova N.A.Copyright ©; 2022, Хорольский Д.В., Клименко А.А., Кондрашов А.А., Шостак Н.А., Демидова Н.А.2022Khorolsky D.V., Klimenko A.A., Kondrashov A.A., Shostak N.A., Demidova N.A.Хорольский Д.В., Клименко А.А., Кондрашов А.А., Шостак Н.А., Демидова Н.А.https://creativecommons.org/licenses/by/4.0https://klinitsist.abvpress.ru/Klin/article/view/513

Systemic scleroderma (SSD) is a rare immune-inflammatory systemic disease of connective tissue with a typical lesion of skin, blood vessels, musculoskeletal system and internal organs (lungs, heart, digestive tract, kidneys). The SSD pathogenesis is based on activation of a cascade of complex immune interactions that lead to vasculopathy. The presence of many pathophysiological links in the progression of the disease causes a variety of clinical manifestations in various patients with SSD. A full assessment of all stages of SSD development is still being carried out and every newly open element of the interaction of immunological subjects completes the overall picture of the disease. A number of studies show a correlation between level of several biomarkers and both disease prognosis and estimated therapy effectiveness. Recent data confirm importance of the biomarkers for formation of patterns of a particular disease phenotype in a specific patient. Depending on relation of the biomarkers to various biological processes, several of their categories are distinguished: biomarkers expressed in lung tissue, cellular units of immunity, nucleic acids, acute phase indicators, connective tissue growth factors, matrix proteinases and their inhibitors, chemokines and cytokines, as well as biomarkers of endothelial activation. Discovery of a novel set of the indicators can be decisive in determining the management tactics and forecasting the response to therapy of some groups of patients with SSD. By combining the most recent data on significant markers obtained in the framework of extensive studies, we have described the most significant biomarkers of SSD and their link to interstitial lung disease (ILD) that is formed in SSD.

Системная склеродермия (ССД) – редкое иммуновоспалительное системное заболевание соединительной ткани с характерным поражением кожи, сосудов, опорно-двигательного аппарата и внутренних органов (легкие, сердце, пищеварительный тракт, почки). В основе ССД лежит активация каскада комплексных иммунных взаимодействий, которые влекут за собой васкулопатию. Разнообразие клинических проявлений ССД у различных пациентов обусловлено множеством патофизиологических звеньев прогрессирования заболевания. Полная оценка всех этапов развития ССД проводится до сих пор, и каждый новый открытый элемент взаимодействия иммунологических субъектов достраивает общую картину заболевания. В ряде исследований показана корреляция между отдельными биомаркерами и их уровнями и прогнозом болезни и предположительной эффективностью терапии. Последние данные подтверждают важность для формирования паттернов того или иного фенотипа заболевания у конкретного пациента. В зависимости от участия биомаркеров в различных биологических процессах выделяют несколько их категорий: биомаркеры, экспрессируемые в легочной ткани, клеточные единицы иммунитета, нуклеиновые кислоты, острофазовые показатели, факторы роста соединительной ткани, матриксные протеиназы и их ингибиторы, хемокины и цитокины, а также биомаркеры эндотелиальной активации. Обнаружение очередного ряда данных показателей может стать решающим в определении тактики ведения и прогнозирования ответа на терапию отдельных групп пациентов с ССД. Мы описали наиболее значимые биомаркеры ССД и их взаимосвязь с интерстициальным заболеванием легких (ИЗЛ), формирующимся при ССД, объединив самые последние данные о значимых маркерах, полученные в рамках обширных исследований.

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