Fig. 5

Cells and cytokines involved in STING-associated autoimmune diseases. Unresolved accumulation of cytoplasmic DNA can potentially trigger chronic inflammatory responses which result in autoimmune diseases, including a systemic lupus erythematosus (SLE) and b Aicardi-Goutières Syndrome (AGS). Both diseases are strongly associated with persistently enhanced type I interferon upregulation named type I interferonopathy and subsequent B and T lymphocyte activation that potentiates systemic tissue and organ damage. Though STING dysregulation has been suggested to play an essential role in the development of these diseases, current treatment of SLE and AGS still relies heavily on anti-inflammatory therapies and DNA resolving methods to ameliorate symptoms. Gain of function mutations of STING can cause two autoimmune diseases named c STING-associated vasculopathy with on-set in infancy (SAVI) and d familial chilblain lupus (FCL). Both diseases show similar manifestations to SLE and AGS and are much less responsive to STING ligands than other immune stimuli. Treatments for SAVI and FCL are limited but JAK inhibitors have been shown to ameliorate symptoms in patients with these two diseases