Open AccessAdult Still's disease: New horizons
Author(s) -
Е. Л. Насонов,
Eugen Feist
Publication year - 2021
Publication title -
naučno-praktičeskaâ revmatologiâ
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.137
H-Index - 9
eISSN - 1995-4492
pISSN - 1995-4484
DOI - 10.47360/1995-4484-2021-643-663
Subject(s) - medicine , immunology , disease , arthritis , proinflammatory cytokine , innate immune system , autoimmunity , pharmacotherapy , inflammation , immune system
Still's disease in children (systemic juvenile idiopathic arthritis - JIA) and adult Still's disease (ASD) are considered as systemic autoinflammatory diseases of unknown etiology, which are based on similar immunopathogenetic mechanisms associated with genetically determined disorders of the mechanisms of innate immunity. ASD was first described 50 years ago by the English rheumatologist Eric George Lapthorne Bywaters. The molecular basis of ASD immunopathogenesis is the activation of innate immunity associated with NLRP3 inflammasome-dependent mechanisms of inflammation, characterized by the overproduction of “pro-inflammatory” cytokines - interleukin (IL) 1 and IL-18, inducing the synthesis of other proinflammatory inflammatory mediators. A review of new data concerning the mechanisms of immunopathology, clinical polymorphism, laboratory biomarkers and the possibilities of ASD pharmacotherapy is presented. Particular attention is paid to the prospects for the use of monoclonal antibodies to IL-1β - canakinumab. The problems associated with the generality of clinical and laboratory disorders, pathogenetic mechanisms and pharmacotherapy of ASD and coronavirus disease 2019 (COVID-19) are considered.