Dr. Crowe and colleagues published data supporting the use of an Interferon Score (IS) to diagnose patients with type I interferonopathies and determine the efficacy of therapeutics for these inflammatory disorders. These assays can identify the relationship between type I interferonopathies and interferon stimulated genes (ISGs) to develop an interferon score that alerts clinicians to the immunological mechanisms of interferonopathies. 

Interferon Signature (IS)

Certain mendelian disorders are associated with upregulation of IFN production, resulting in inflammatory phenotypes. Disturbances to the homeostatic maintenance of this system can result in illnesses similar to lupus erythematosus (SLE) and dermatomyositis (DM). 

Tulane University

Type I Interferon is a subset of interferon (IFN) that can be divided into multiple categories. However, rheumatologists generally only consider IFN-Beta and IFN-Alpha, which are transcribed by a family of linked genes on chromosome 9. Disruption to IFN signaling can result in inflammatory and autoimmune disorders, which are at times referred to as type I interferonopathies. Functioning immune systems are characterized by low concentrations of circulating type I IFN and cells with type I IFN receptors. 

Type I Interferon (IFN)

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.

Assessment of Type I Interferon Signaling in Pediatric Inflammatory Disease

Type I IFN has anti-viral properties which allow infected cells to develop an intracellular response to a viral infection. Infected cells are oriented to be easily destroyed by killer lymphocytes and IFN alerts nearby cells to the danger. These signaling pathways are performed in an autocrine and paracrine process to induce the transcription of IFN-alpha and activate a signaling response in uninfected cells. 

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