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Abstract Background and aim: Systemic lupus erythematosus (SLE) is a multivariant autoimmune disease resulting from both genetic and environmental factors. The type I IFN pathway is pathologically overactive in many SLE patients. The study aimed to study the impact of IFIH1 gene SNP on interferon pathway signaling in SLE patients specifically with vascular affection. Subjects and methods: 30 consecutive patients known to have SLE according to the ACR compared to 10 healthy age and sex matched controls regarding QRT- PCR gene expression of IFIH 1, vWF (von Willibrand factor, IMT & ABI Results: IFIH1 was significantly higher in patient than in controls (1.7+0.6 and 0.49+0.21) respectively with (P.value =0.0001). At a cutoff value 0.91 IFIH1 had 96% sensitivity and 100% specificity as a lupus associated gene. Conclusion: IFIH1 gene (SNP 1990670) is associated with SLE in the egyptian patients, it is related to the disease activity and can serve as a predictor of vascular affection in SLE |