nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.24884/1561-6274-2012-16-2-49-63nefr-579Research ArticleОБЗОРЫ И ЛЕКЦИИREVIEWS AND LECTURESАУТОВОСПАЛИТЕЛЬНЫЕ ЗАБОЛЕВАНИЯ: ОБЩЕЕ ПОНЯТИЕ, МЕХАНИЗМЫ РАЗВИТИЯ, КЛИНИЧЕСКАЯ КАРТИНА, ПОДХОДЫ К ЛЕЧЕНИЮAUTOINFLAMMATORY DISEASES: GENERAL IDEA, DEVELOPMENT MECHANISMS, CLINICAL PRESENTATION, TREATMENTРамеевВ. В.RameevV. V.

Кафедра нефрологии, внутренних и профессиональных заболеваний им.Е.М.Тареева 

Тел.: 89104936138

vvrameev@mtu-net.ruКозловскаяЛ. В.KozlovskayaL. V.

Кафедра нефрологии, внутренних и профессиональных заболеваний им.Е.М.Тареева

Первый МГМУ им.И.М.СеченоваRussian Federation2012100220121624963Copyright © Рамеев В.В., Козловская Л.В., 20122012Рамеев В.В., Козловская Л.В.Rameev V.V., Kozlovskaya L.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephrolog.ru/jour/article/view/579

В последние годы в медицинской научной литературе появилось новое понятие «системные аутовоспалительные заболевания». В отличие от аутоиммунных заболеваний в инициации аутовоспалительных болезней играют роль, главным образом, генетически детерминированные реакции иммунитета и воспаления, а не механизмы, связанные с антигениндуцированным синтезом антител или активацией Т-лимфоцитов. Группа аутовоспалительных заболеваний включает в себя семейные периодические лихорадки (периодическую болезнь, синдром Макла–Уэллса и др.), наиболее частым осложнением которых является вторичный АА-амилоидоз. Молекулярно-генетические исследования показали, что важнейшим механизмом аутовоспаления является нейтрофильное воспаление, ассоциированное с гиперпродукцией интерлейкина-1. Ведущим пусковым механизмом нейтрофильного воспаления является образование макромолекулярного комплекса – криопириновой инфламмасомы, которая имеет важное значение в инициации воспаления не только при семейных периодических лихорадках, но и при ряде широко распространенных воспалительных заболеваний (подагра, ХОБЛ и др.). В этой связи криопириновая инфламмасома может рассматриваться как один из универсальных механизмов нейтрофильного воспаления. Хронические аутовоспалительные заболевания создают персистирующий воспалительный фон и способствуют активации иммунных реакций с возможным развитием классических аутоиммунных заболеваний, что наглядно показывают представленные клинические наблюдения.

During last years in medical literature appeared new term “systemic autoinflammatory diseases”. In contrast to autoimmune diseases in autoinflammatory diseases initiation the main role belongs not to mechanisms associated with antigen-induced synthesis of antibodies or T-cells activation but allelically determined reactions of immunity and inflammatory. The group of autoinflammatory diseases includes familiar periodic fevers (Mediterranian fever, Muckle–Wells syndrome etc.) which most frequent complication is recurrent AA-amyloidosis. DNA testing showed that the most important mechanism of autoinflammation is neutrophilic inflammation associated with interleukin-1 hyper production. The main releaser of neutrophilic inflmmation is development of macromolecular complex – NLRP3 inflammasome which is very important in inflammation initiation not only at familiar periodic fevers but also at a number of widespread inflammatory diseases (gout, COPD etc.). In this regard NLRP3 inflammasome can be considered as one of universal mechanisms of neutrophilic inflammation. Chronic inflammatory diseases make persistant background and promote activation of immune reactions with possible development of classical autoimmune diseases that presented clinical studies demonstrate.

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