340 IS were measured from 207 patients with undiagnosed mutated genotypes. However, the data implied upregulation of IFN signaling in a large proportion of patients with juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM). Alternatively, a lower percentage of patients with autoinflammatory phenotypes (without a molecularly determined mutated genotype) presented a positive IS. 

Tulane University

Patients in group III included those diagnosed with juvenile SLE; juvenile DM; systemic juvenile idiopathic arthritis (sJIA) and non-systemic JIA; autoinflammatory disorders with no molecularly determined diagnosis; and patients with neurological, dermatological and rheumatological symptoms consistent with type I interferonopathies. 

Group III

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

Parham, P. (2009). Vaccination to Prevent Infectious Disease. In 1145902806 862124677 J. Foltin (Ed.), The Immune System (3rd ed., pp. 62-65). New York, New York: Garland Science, Taylor & Francis Group.

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