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We thank Banday et al for their correspondence and their concise summary of information about monogenic interferonopathies and the response to various Jak inhibitors.1 As we have detailed in the paper, the Jakinib consensus statement and the associated systematic literature research2 3 focused on:
Practical information for the clinician to assist with Jakinib therapy once the decision is made to prescribe a Jakinib no matter which disease indication exists such as those discussed in the correspondence.
Entailed strict criteria for study inclusion which in particular excluded small cohort studies such as those quoted in the correspondence, moreover.
Off label indications for Jakinib use such as the monogenic interferonopathies were also excluded pending future high quality randomised clinical trials (RCTs) for peer-reviewed assessment of Jakinib therapy, although understandably this is difficult in orphan diseases that are rare and meaningful numbers to study the efficacy and safety of any therapy is a real challenge.
With respect to the interferonopathies, we cited three publications, including both sting-associated vasculopathy infantile onset (SAVI) syndrome and USP18 disease which are regarded as interferonopathies as mentioned by Banday et al
In line with the conclusion in the correspondence that ‘the data are still not plentitude’, we look forward to future developments in this field and hope that these developments will lead to approval of Jakinibs for interferonopathies in the near future.
Peter Nash and Josef S Smolen on behalf of the Jakinib Consensus group.
Handling editor David S Pisetsky
Contributors The work is mine and JSS’s work.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.
Patient consent for publication Not required.
Provenance and peer review Commissioned; internally peer reviewed.
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