Autoinflammatory syndrome

New research has identified an autoinflammatory syndrome caused by mutations in NEMO-encoding IKBKG, leading to NF-κB activation and type I interferon production.

Chromatin undergoes extensive reprogramming during immune cell differentiation. Histone clipping, an underexplored epigenetic mechanism, ensures precise macrophage development and function and is now found to be dysregulated in autoinflammatory disease.

Two new studies have identified mutations in UBA1 associated with the newly identified condition VEXAS syndrome in cohorts of patients with relapsing polychondritis, supporting the concept that relapsing polychondritis is more than one disease.

The ubiquitin-editing enzyme A20 has a pivotal role in restricting autoimmune and inflammatory responses. New studies suggest that A20 prevents inflammatory diseases using a non-catalytic mechanism involving ubiquitin binding.