The elusive pathogenesis of Schnitzler syndrome

Giovanni Palladini and Giampaolo Merlini

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  • RE: The elusive pathogenesis of Schnitzler syndrome
    • Sujoy Khan, Consultant Immunologist Apollo Gleneagles Hospital, 58 Canal Circular Road, Kolkata

    Dear Editor,
    The fascinating paper and commentary on elucidation of mechanisms of Schnitzler syndrome where search for mutations in NLRP3 and 32 other genes failed to show a monogenic cause [1]. We recently reported a variant Schnitzler syndrome (IgG clone) who responded to colchicine (no fevers, complete disappearance of rash, but joint pains persist) as Anakinra could not be obtained [2], suggesting that microtubule inhibition only partially works in the autoinflammatory syndromes. That anakinra works in Schnitzler syndrome even without mutations in NLRP3 inflammasome is similar to its efficacy seen in children with CIAS1 gene mutation negative CINCA/NOMID (chronic infantile neurologic cutaneous articular / neonatal-onset multisystem inflammatory disease) [3], indicates that IL-1 receptor blockade on a wide range of cells and tissues ‘switches off’ (temporarily) the inflammasome.

    Where then is the pathogenic mutation that increases ASC, IL-18 and IL-6 levels? Activation of the inflammasome is the only way to also explain the high bone alkaline phosphatase (bALP), osteocalcin, osteoprotegerin and VEGF levels seen in most patients. A world-wide registry is definitely required to collaborate between physicians and scientists with a centrally located facility that would accept patient samples to continue to search for an answer for this elusive disorder.


    1. Rowczenio DM, Pathak S, Arostegui JI, Mensa-Vilaro A, Omoyinmi E, Brogan P et...

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    Conflict of Interest:
    None declared.