Dramatic Efficacy of Ibrutinib in a Schnitzler Syndrome Case with Indolent Lymphoma

Excerpt

Schnitzler syndrome (SchS) is a rare auto-inflammatory disorder first described in 1972 whose diagnosis relies on the presence of two major criteria, IgM or IgG monoclonal immunoglobulin detection and chronic urticarial rash, and several minor criteria: recurrent fever, objective findings of abnormal bone remodeling with or without bone pain, a neutrophilic dermal infiltrate on skin biopsy, leukocytosis, and/or elevated CRP. Until now, little was known about its physiopathology which is suspected to involve the inflammasome and interleukin 1 Beta (IL-1β) pathway [1].The introduction of recombinant anti-IL-1 receptor (IL-1R) (anakinra) in a therapeutic strategy drastically improved the quality of life of these patients, but relapses are frequent shortly after the withdrawal of IL-1 blockade or during treatment. The main complication of SchS is the development of indolent lymphoproliferative disorders in about 20% of cases during its evolution [2]. As IL-1 blockade only curbs symptoms of SchS and since resistance may appear after some years, alternative treatments represent a medical need, particularly in patients with only partial response or treatment resistance. …