What question did this study set out to answer?

This research aims to explore the connection between the STING pathway and interstitial lung disease, particularly in SAVI and COPA syndrome.

June 3, 2026Journal of Clinical Investigation0 citationsOpen Access

Interstitial lung disease and the STING pathway

PVPrasad Palani VeluEdinburgh Royal Infirmary GZGaofeng Zhu KMKaren J. MackenzieGoogle (United States)

Key Points

This research aims to explore the connection between the STING pathway and interstitial lung disease, particularly in SAVI and COPA syndrome.
Overview of STING-associated vasculopathy with onset in infancy (SAVI) and coatomer protein complex subunit alpha (COPA) syndrome.
Review of current evidence on STING activity in ILD pathogenesis and related model systems.
Discussion on the therapeutic implications of targeting the STING pathway in common forms of ILD.
Identified genetic mutations in SAVI and COPA syndrome are linked to dysregulation of the STING pathway.
STING pathway involvement suggests potential for targeted therapeutics in rare and possibly more common ILDs.
Current data provides insight but lacks clarity regarding the relevance of STING in broader ILD contexts.

Abstract

Identification of the genetic mutations underlying the ultrarare monogenic conditions STING-associated vasculopathy with onset in infancy (SAVI) and coatomer protein complex subunit alpha (COPA) syndrome revealed a role for the stimulator of interferon genes (STING) immune pathway in the pathogenesis of interstitial lung disease (ILD) in these conditions. STING-focused therapeutics could be a potential avenue for the treatment of SAVI and COPA syndrome in the future, yet the relevance of STING to more common types of ILD is not clear. Here, we provide an overview of SAVI and COPA syndrome, the nature of ILD in these conditions, and current evidence regarding STING activity in their pathogenesis. We discuss data from studies of a variety of other ILDs and model systems and explore the potential role for STING in more common forms of ILD.

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