TY - JOUR T1 - Response to: ‘JAK inhibition in STING-associated interferonopathy’ by Crow <em>et al</em> JF - Annals of the Rheumatic Diseases JO - Ann Rheum Dis SP - e76 LP - e76 DO - 10.1136/annrheumdis-2016-210565 VL - 75 IS - 12 AU - Victoria Tüngler AU - Nadja König AU - Claudia Günther AU - Kerstin Engel AU - Christoph Fiehn AU - Martin Smitka AU - Maja von der Hagen AU - Reinhard Berner AU - Min Ae Lee-Kirsch Y1 - 2016/12/01 UR - http://ard.bmj.com/content/75/12/e76.abstract N2 - 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 … ER -