Type 1 interferons (IFNs) have a crucial role in the innate immune system’s protection against viral infections. The production of type 1 IFNs is triggered by a signalling pathway that is activated when innate sensors recognise viral nucleic acids. Type 1 IFNs bind to the receptors IFNAR1 and IFNAR2, thereby initiating JAK-STAT signalling, which promotes the expression of IFN-stimulated genes and drives the inflammatory response. Monogenic autoinflammatory disorders that lead to dysregulation of the type 1 IFN response—either through excessive activation or impaired downregulation—are collectively known as type 1 interferonopathies.