A group of 24 patients from 17 different families produced a positive interferon score (IS) consistent with the upregulation of type I interferon associated with type I interferonopathies. However, these patients tested negative for any type I interferonopathy associated genes. Some of these patients may have genetically undetermined causative factors of the clinical phenotype, warranting additional investigation. 

Tulane University

A majority of patients in groups II and III were receiving immunosuppression therapy. However, most patients with diagnoses of JSLE and JDM presented with persistent upregulation of interferon signaling regardless of the immunosuppressive treatment. Certain patients with sJIA did present with upregulated ISG expression even with IL1 blockers. Additionally, the interferon score should not be mistaken for other sensitive assays that can identify the disease-inducing molecule. The IS relates to the expression of ISGs and can be used, in conjunction with other technology, to describe the relationship between the inducing signal and the subsequent response. 

Limitations of the Crowe Study

Rice GI, Melki I, Frémond ML, Briggs TA, Rodero MP, Kitabayashi N, Oojageer A, Bader-Meunier B, Belot A, Bodemer C, Quartier P, Crow YJ. Assessment of Type I Interferon Signaling in Pediatric Inflammatory Disease. J Clin Immunol. 2017 Feb;37(2):123-132. doi: 10.1007/s10875-016-0359-1. Epub 2016 Dec 9. PMID: 27943079; PMCID: PMC5325846.

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