cardioКардиологияKardiologiia0022-90402412-5660Kardiomag10.18087/cardio.2021.1.n1475cardio-1475Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИRESEARCH ARTICLESИдиопатический рецидивирующий перикардит – новое орфанное аутовоспалительное заболевание? Ретроспективный анализ случаев идиопатического рецидивирующего перикардита и план рандомизированного, плацебо-контролируемого исследования для оценки терапевтической эффективности и безопасности блокатора ИЛ-1 (RPH-104)Idiopathic recurrent pericarditis – a new orphan autoinflammatory disease? A retrospective analysis of cases of idiopathic recurrent pericarditis and a design of а double-blind, randomized, placebo-controlled study to evaluate the efficacy and safety of RPH-104 treatment in patients with idiopathic recurrent pericarditishttps://orcid.org/0000-0002-7590-742XМячиковаВ. Ю.MyachikovaV.  Yu.

врач-ревматолог, младший научный сотрудник НИЛ ревматологии и иммунопатологии

rheumatologist, junior science worker of laboratory of rheumatic and immunopathology

myachik9191@gmail.comhttps://orcid.org/0000-0003-2427-4148МаслянскийА. Л.MaslyanskiyA. L.

врач-ревматолог, д.м.н., заведующий НИЛ ревматологии и иммунопатологии

head of laboratory of rheumatic and immunopathology

esc_4@mail.ruhttps://orcid.org/0000-0002-7817-3847МоисееваО. М.MoiseevaO. M.

врач-кардиолог, д.м.н, профессор, директор института сердца и сосудов, главный научный сотрудник научно-исследовательский отдел  некоронарогенных заболеваний сердца

prof, Director of the Heart and Vascular Institute ,Head of laboratory Non-Coronary Heart Diseases

moiseeva.cardio@gmail.comФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, Санкт-ПетербургРоссияAlmazov National Medical Research Centre, St. Petersburg, RussiaRussian Federation2021100220216117277Copyright © Kardiomag, 20212021KardiomagKardiomaghttps://cardio.elpub.ru/jour/about/submissions#copyrightNoticehttps://cardio.elpub.ru/jour/article/view/1475

Цель Анализ случаев идиопатического рецидивирующего перикардита (ИРП) в структуре заболеваний перикарда различного генеза по обращаемости в многопрофильный федеральный центр.Материал и методы Проведен ретроспективный анализ историй болезни пациентов, госпитализированных в ФГБУ «НМИЦ им В. А. Алмазова» за период с 1 января 2015 года по 1 января 2020 года с выпотом в полость перикарда различной этиологии.Результаты За обозначенный период выявлен 4 981 новый случай поражения перикарда различной этиологии, среди которых на постперикардиотомный синдром приходилось 4 360 всех случаев, а на долю перикардитов – 621 случай. ИРП выявлен в 34 случаях, что составило 5,4 %. С учетом полученных данных расчетный показатель распространенности ИРП в Российской Федерации может составлять 1,1 случая на 100 тыс. населения.Заключение ИРП следует рассматривать, как новое аутовоспалительное заболевание, распространенность которого граничит с таковой при болезни Стилла взрослых и должна рассматриваться в концепции орфанных патологий. Современные знания о патогенезе и данные последних исследований демонстрируют высокую значимость блокады интерлейкина-1 в качестве ведущего механизма достижения ремиссии, что стало обоснованием к проведению рандомизированного клинического исследования на базе Центра.

Aim To analyze cases of idiopathic recurrent pericarditis (IRP) in the structure of pericardial diseases of various origins from patient visits to the Multidisciplinary Federal Center.Material and methods A retrospective analysis of case records was performed for patients admitted to the V.A. Almazov National Medical Research Center from January 1, 2015 through January 1, 2020 for pericardial effusion of different etiologies.Results For the study period, 4 981 new cases of pericardial damage of different etiologies were found. Among these cases, postpericardiotomy syndrome accounted for 4 360 cases and pericarditis for 621 cases. IRP was detected in 34 cases, which amounted to 5.4 %. Based on the study data, the estimated IRP prevalence in the Russian Federation can be 1.1 cases per 100 thousand population.Conclusion IRP should be regarded as a new autoinflammatory disease, the prevalence of which borders on that of adult Still disease and should be addressed within the concept of orphan diseases. Current knowledge of the pathogenesis and data from recent studies demonstrated a great importance of interleukin-1 blockade as a leading mechanism for achieving remission. This has justified conduction of a randomized clinical study at the Center.

Идиопатический рецидивирующий перикардитэпидемиологияпатогенезорфанные заболеванияаутовоспалительные заболеванияинфламмасомаинтерлейкин-1клиническое исследованиеблокатор интерлейкина 1 бетаIdiopathic recurrent pericarditisepidemiologypathogenesisorphan diseasesautoinflammatory diseasesinflammasomeinterleukin-1clinical studyinterleukin-1β blockerКонфликт интересов не заявлен.No conflict of interest is reported.ReferencesKastner DL. Autoinflammation: Past, Present, and Future. In: Textbook of Autoinflammation. P. 3-15 [DOI: 10.1007/978-3-319-98605-0_1]. Hashkes PJ, Laxer RM, Simon A, editors -Cham: Springer International Publishing;2019. ISBN 978-3-319-98604-3Kastner DL. Autoinflammation: Past, Present, and Future. In: Textbook of Autoinflammation. P. 3-15 [DOI: 10.1007/978-3-319-98605-0_1]. Hashkes PJ, Laxer RM, Simon A, editors -Cham: Springer International Publishing;2019. ISBN 978-3-319-98604-3Masters SL, Simon A, Aksentijevich I, Kastner DL. 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The authors declare that there are no conflicts of interest present.