26rhk150 10.3205/26rhk150 urn:nbn:de:0183-26rhk1507 Meeting Abstract Rittich Rittich Emma E

Department of Pediatric Immunology, Rheumatology and Infectiology, Hospital for Children and Adolescents, University Hospital Leipzig, Leipzig, Deutschland

author Hohmann Hohmann Tamara T

Department of Pediatric Immunology, Rheumatology and Infectiology, Hospital for Children and Adolescents, University Hospital Leipzig, Leipzig, Deutschland

author Klemann Klemann Christian C

Department of Pediatric Immunology, Rheumatology and Infectiology, Hospital for Children and Adolescents, University Hospital Leipzig, Leipzig, Deutschland

author German Medical Science GMS Publishing House

Düsseldorf

610 20260909 engl This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). M0656 150 Deutsche Gesellschaft für Rheumatologie Deutsche Gesellschaft für Orthopädische Rheumatologie Gesellschaft für Kinder- und Jugendrheumatologie 54. Kongress der Deutschen Gesellschaft für Rheumatologie und Klinische Immunologie (DGRh), 36. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR), 40. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh) Deutscher Rheumatologiekongress 2026 Kinderrheumatologie Leipzig 20260909 20260912 KI.07 TextIntroduction: STING-associated vasculopathy with onset in infancy (SAVI) is a prototypic type I interferonopathy caused by activating STING1/TMEM173 variants and frequently complicated by interstitial lung disease (ILD). Comparative treatment data remain limited.Methods: We reviewed 133 published SAVI literature patients for treatment response across therapeutic classes and report one infant with genetically confirmed SAVI (STING1 p.Arg281Trp, homozygous) and severe early-onset ILD treated with ruxolitinib followed by add-on anifrolumab.Results: Glucocorticoids provided mainly transient control with frequent steroid dependence, whereas conventional DMARDs and non-pathway-specific biologics were largely ineffective. Among targeted therapies, ruxolitinib showed 12/29 improved, 9/29 partial/mixed, and 8/29 failed responses. Baricitinib showed 12/27 improved, 9/27 partial/mixed, 3/27 failed responses, and 3/27 without sufficient information on the outcome. Tofacitinib was less consistent, with 6/16 improved, 4/16 partial/mixed, and 6/16 failed responses. Anifrolumab showed improvement in 6/6 reported exposures, although numbers remain small. Lung disease remained the most difficult organ manifestation, with stabilization more common than reversal once fibrosis was established. Our infant presented in the first year of life, before severe fibrosis had developed, with tachypnoea, increased work of breathing, failure to thrive, and nocturnal home oxygen dependence. Ruxolitinib started at 8 months and produced no measurable clinical improvement. After add-on anifrolumab, monocyte CD169/SIGLEC1 rapidly fell from ~8,600–13,000 antigen/cell to ~1,100, paralleled by improved respiratory rate, oxygen discontinuation, and catch-up growth.Conclusion: Conventional immunosuppression is largely inadequate in SAVI, whereas pathway-directed treatment is required. Overall, baricitinib and ruxolitinib showed the strongest treatment signal, tofacitinib was less reliable, and IFNAR1 blockade emerged as a promising strategy. Our review and original case support further evaluation of anifrolumab, especially early, before fixed pulmonary fibrosis is established. 0 0 0 0