Innately adaptive or truly autoimmune.

Kessel, Christoph, Hedrich, Christian M ORCID: 0000-0002-1295-6179 and Foell, Dirk
(2019) Innately adaptive or truly autoimmune. Arthritis & rheumatology (Hoboken, N.J.), 72 (2). pp. 210-219.

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Systemic juvenile idiopathic arthritis (sJIA) is a form of arthritis in childhood that is initially dominated by innate driven systemic inflammation and is thus considered a polygenic autoinflammatory disease. However, sJIA can progress towards an adaptive immunity driven afebrile arthritis. Based on this observation of bi-phasic disease progression, a "window-of-opportunity" for optimal, individualized and target-directed treatment has been proposed. This hypothesis requires testing and in this review we summarize current evidence regarding molecular factors that may contribute to the progression from an initially predominantly autoinflammatory disease phenotype to autoimmune arthritis. We consider the involvement of innately adaptive γδT and NKT cells that express γδ or αβ T cell receptors but can be neither classified as purely innate or adaptive cells, versus classical B and T lymphocytes in this continuum. Finally, we discuss our understanding of how and why some primarily autoinflammatory conditions can progress towards autoimmune-mediated disorders over the disease course while others do not and how this knowledge may be used to offer individualized treatment.

Item Type: Article
Uncontrolled Keywords: Humans, Autoimmune Diseases, Inflammation, Immunity, Innate, Adaptive Immunity, Arthritis, Juvenile
Depositing User: Symplectic Admin
Date Deposited: 26 Sep 2019 14:16
Last Modified: 19 Jan 2023 00:25
DOI: 10.1002/art.41107
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