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Correspondence response
Response to: ‘JAK inhibition in STING-associated interferonopathy’ by Crow et al
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  1. Victoria Tüngler1,
  2. Nadja König1,
  3. Claudia Günther2,
  4. Kerstin Engel1,
  5. Christoph Fiehn3,
  6. Martin Smitka4,
  7. Maja von der Hagen4,
  8. Reinhard Berner5,
  9. Min Ae Lee-Kirsch1
  1. 1Molecular Pediatrics, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
  2. 2Department of Dermatology, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
  3. 3ACURA Akutklinik für Rheumatologie Baden-Baden, Baden-Baden, Germany
  4. 4Abteilung Neuropaediatrie, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
  5. 5Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
  1. Correspondence to Professor Min Ae Lee-Kirsch, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Fetscherstr. 74, 01307 Dresden, Germany; minae.lee-kirsch{at}uniklinikum-dresden.de

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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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    Mathieu P Rodero Marie-Louise Frémond Gillian I Rice Bénédicte Neven Yanick J Crow