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Significance STING is a key driver of monogenic interferonopathies that include STING-associated vasculopathy with onset in infancy (SAVI). Defining the molecular mechanisms and the contribution of individual cell types to disease could unveil new therapeutic targets for management of SAVI and related autoimmune diseases. Here we compare and contrast murine models representative of the two most common mutations and identify a hierarchy of lymphoid abnormalities that depend on T cell-intrinsic expression of the SAVI mutation. Although clinical manifestations result from hyperactivation of STING, they occur independently of type I interferon and necroptosis. These murine models facilitate a dissection of the role of STING in specific tissues and provide tools for evaluating STING inhibitors for the treatment of SAVI patients.

Hierarchy of clinical manifestations in SAVI N153S and V154M mouse models