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We appreciate the comments by Crow et al on our report of a gain-of-function mutation in STING in familial chilblain lupus and the effect of the JAK inhibitor tofacitinib.1 In a concurrent study,2 Crow et al reported a marked clinical improvement in three patients with stimulator of interferon genes (STING)-associated vasculopathy3 using the JAK inhibitor ruxolitinib. This was accompanied by an incomplete reduction in the expression of interferon (IFN)-stimulated genes (ISGs) in blood raising the question as to the IFN signature as a read-out of clinical efficacy. This is a very important question that we cannot answer with regard to tofacitinib as we have no long-term experience with this JAK inhibitor in patients with type I interferonopathies. However, we have used ruxolitinib in two patients with Aicardi-Goutières syndrome (AGS), an inflammatory encephalopathy characterised by chronic type I IFN activation.4 Both patients carried biallelic RNASEH2B mutations and suffered from severe developmental delay. They received no other medications, when treatment with ruxolitinib …
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