Schnitzler syndrome: response to anakinra in two cases and a review of the literature.
BACKGROUND
Schnitzler syndrome is a rare disease characterized by a chronic urticarial eruption and monoclonal gammopathy, as well as clinical and laboratory signs of inflammation. The pathophysiology is still unknown, although various autoantibody-mediated mechanisms have been described. Complete remission of symptoms has been reported recently in patients with Schnitzler syndrome treated with anakinra, an interleukin-1 receptor antagonist.


METHODS
Two patients with Schnitzler syndrome treated with anakinra therapy are presented.


RESULTS
We report two cases of nearly complete remission of symptoms in Schnitzler syndrome after the initiation of anakinra therapy, and the first observation of a relapse under continuous daily anakinra therapy. A review of the published literature on the treatment of Schnitzler syndrome with anakinra is presented.


CONCLUSIONS
Based on published data, monotherapy with anakinra is currently the most promising treatment for Schnitzler syndrome, because it is able to induce complete remission of symptoms.
Schuster
Christian
C
Department of Environmental Dermatology and Allergy, Medical University of Graz, Graz, Austria. christian.schuster@meduni-graz.at
Kränke
Birger
B
Aberer
Elisabeth
E
Arbab
Emad
E
Sturm
Gunter
G
Aberer
Werner
W
D000328:Adult; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012074:Remission Induction; D019873:Schnitzler Syndrome / Q000188:drug therapy*; D016896:Treatment Outcome
D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein