{"doi":"10.1002/art.43145","title":"Sustained Interferon Signature Suppression With Anifrolumab in a Patient With STING‐Associated Vasculopathy with Onset in Infancy Refractory to <scp>JAK</scp> 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 monitored throughout both treatment periods. RESULTS: Despite initial reductions in C-reactive protein levels and IFN I scores following dazukibart administration, the patient experienced rebound inflammation and recurrent vasculitic lesions. Dazukibart dose adjustments failed to sustainably control IFN I signaling. Subsequent combination therapy of baricitinib and tocilizumab proved partially effective. Treatment with anifrolumab, an IFNAR1 blocker, in conjunction with tocilizumab led to sustained suppression of IFN I scores, allowed discontinuation of JAKi, and resulted in significant improvement in vasculitic wounds. CONCLUSION: This case underscores the challenges in treating patients with SAVI and highlights the utility of IFN I scores as a theragnostic biomarker. Although high-dose JAKi and dazukibart failed to achieve sustained control of IFN I signaling, treatment with anifrolumab durably suppressed IFN scores and demonstrated promising efficacy, which allows for the investigation of the role of IFN I signaling in the disease pathogenesis of SAVI and other interferonopathies in future clinical trials.","journal":"Arthritis & Rheumatology","year":2025,"id":511732,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":14,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9602,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2025-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1240685,"name":"Bjoern Buehring","orcid":"0000-0003-3841-624X","position":1,"is_corresponding":false},{"id":104156,"name":"Adriana A. de Jesus","orcid":"0000-0001-8966-8362","position":2,"is_corresponding":false},{"id":1371099,"name":"Sachin Gaurav","orcid":null,"position":3,"is_corresponding":false},{"id":231240,"name":"Andre Rastegar","orcid":"0000-0003-4724-9041","position":4,"is_corresponding":false},{"id":1371100,"name":"Alexi Baumgardner","orcid":null,"position":5,"is_corresponding":false},{"id":1350491,"name":"Kip Friend","orcid":"0009-0000-3277-026X","position":6,"is_corresponding":false},{"id":1022316,"name":"Oluwatobi Arisa","orcid":"0000-0001-6668-3105","position":7,"is_corresponding":false},{"id":109200,"name":"William D. Figg","orcid":"0000-0003-2428-5613","position":8,"is_corresponding":false},{"id":231229,"name":"Danielle Fink","orcid":"0000-0002-7502-7385","position":9,"is_corresponding":false},{"id":231246,"name":"Douglas B. Kuhns","orcid":"0000-0002-9971-0760","position":10,"is_corresponding":false},{"id":1203384,"name":"Ben Colton","orcid":null,"position":11,"is_corresponding":false},{"id":109199,"name":"Cody J. Peer","orcid":"0000-0001-9395-3473","position":12,"is_corresponding":false},{"id":104223,"name":"Raphaela Goldbach‐Mansky","orcid":"0000-0001-7865-5769","position":13,"is_corresponding":false},{"id":231231,"name":"Sara Alehashemi","orcid":"0000-0002-6531-6108","position":0,"is_corresponding":true}],"reference_count":11,"raw_metadata":null,"created_at":"2026-07-19T02:48:01.269605Z","pmid":"40007227","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}