Sustained Interferon Signature Suppression With Anifrolumab in a Patient With STING-Associated Vasculopathy with Onset in Infancy Refractory to JAK Inhibitor and Dazukibart Therapy.

ABSTRACT:

Objective

The objective was to report the safety and efficacy of an anti-IFNAR1 antibody (anifrolumab) in a patient with STING-associated vasculopathy with onset in infancy (SAVI) who presented with vasculitic ulcers and systemic inflammation refractory to JAK inhibition (JAKi) and to the interferon-β-neutralizing monoclonal antibody dazukibart.

Methods

A patient with SAVI and a de novo STING1 p.(Asn154Ser) mutation, a known pathogenic variant, and uncontrolled disease received 21 doses of dazukibart under a compassionate use investigational new drug protocol, which was followed by treatment with the anti-IFNAR1 antibody anifrolumab. Clinical and laboratory parameters, including wound healing, whole-blood type I interferon (IFN I) signature, and safety markers were closely m

SUBMITTER: Alehashemi S 

PROVIDER: S-EPMC12311253 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature