In the IUIS-Immunopaedia-Frontiers in Immunology Webinar he gave examples of clinical presentations and laboratory features of hyper-inflammation with a focus on hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS). He also discussed Pathogenic mechanisms and the involvement of cytokine networks in different forms of hyper-inflammation, as well as conventional and novel targeted therapeutic approaches. Highlights of his talk include:
- Brief on the definitions and examples of autoimmunity (triggered by adaptive immunity), auto-inflammation (triggered by adaptive immunity) and hyper-inflammation (triggered by innate and adaptive immunity).
- He also gave an overview on COVID-19 immunopathology includes hyper-inflammation.
- Mechanism of HLH : prolonged T cell activation which activates cytokine storm via the overproduction of IFN-g, and the potential treatment of paediatric HLA using anti-IFNg (emapalumab in clinical trial testing) (Locatelli et al. NEJM). Findings by Locatelli et al. demonstrated a good complete (26%) and partial (30%) response rate.
- IFNg is not the only molecule involved in HLH, he also provided data that highlighted a role of IL-2 and IL-33.
- He also gave an overview of how paediatric rheumatoid arthritis and HLA are associated with CXCL9 and IFNg induced hyperinflammation, and IL-6 and IL-18 activated MAS.
- Finally, he gave multiple examples of the clinical potential of IFNg blockade in treatment of HLH and MAS.
- Summary of all talking points of the webinar are below.
