How autoinflammation may turn into autoimmune inflammation: Insights from monogenetic and complex IL-1 mediated auto-inflammatory diseases

• Published in: Clinical immunology (Orlando, Fla.) Fla.), 2020-10, Vol.219, p.108538-108538, Article 108538
• Main Authors: ter Haar, N.M., Jansen, M.H.A., Frenkel, J.F., Vastert, S.J.
• Format: Article
• Language: English
• Subjects: Auto-immune inflammation; Auto-inflammation; Mechanisms of disease
• ISSN: 1521-6616; 1521-7035
• DOI: 10.1016/j.clim.2020.108538

IL-1 mediated auto-inflammatory diseases are characterised by episodes of unexplained fever, generalized and localized inflammation. The characteristic symptoms predominantly result from exaggerated activation of innate immune pathways. However, in some patients with typical IL-1 mediated diseases, chronic disease manifestations develop in the absence of acute inflammation, suggesting the involvement of adaptive immune pathways. We discuss clinical observations as well as novel insights in how chronic activation of innate immune pathways can lead to auto-immune disease features in patients with auto-inflammatory diseases and how we need to better understand these sequelae in order to improve treatment strategies. •IL-1 mediated auto-inflammatory diseases are characterised by episodes of unexplained fever and a variety of episodic organ involvement.•We summarize characteristic symptoms and presumed underlying disease mechanisms, predominantly resulting from exaggerated activation of innate immune pathways.•Patients with typical IL-1 mediated diseases, sometimes chronic and complex disease manifestations develop, suggesting the involvement of adaptive immune pathways.•We discuss clinical observations as well as novel insights in how chronic activation of innate immune pathways can lead to auto-immune disease features in patients with auto-inflammatory diseases and how we need to better understand these sequella in order to improve treatment strategies.