Schnitzler-Syndrom

Abstract

Schnitzler syndrome is a rare systemic inflammatory disease characterized by the presence of chronic urticarial skin rash and a monoclonal immunoglobulin M (IgM) gammopathy, combined with further, variable disease symptoms. The term refers to a young disease entity which has recently gained increasing acknowledgement and attention, also due to the availability of interleukin-1 (IL-1) blockade as an effective therapeutic option. Insights into the pathophysiology of the disease have resulted in the assumption of Schnitzler syndrome being a special form of an autoinflammatory disease with late onset or an acquired genesis. This article provides an overview on the clinical appearance, current knowledge of pathophysiology and available therapeutic options.

Schnitzler's syndrome: lessons from 281 cases

“…To date, 281 cases have been reported, with a male–female ratio of 1.5 [2–146]. (Kabashima, Schiff, Frerichs, Van Hoof, Relas, Björk, Fox, Croot: personal communications (p.c.))…”

VEXAS-Syndrom

“…Interessanterweise tritt jedoch ein Teil der autoinflammatorischen Erkrankungen auch in Assoziation mit hämatologischen Veränderungen oder Erkrankungsbildern bei älteren Patienten auf. Prominentestes Beispiel ist das Schnitzler-Syndrom, welches gekennzeichnet ist durch eine monoklonale Hypergammaglobulinämie vom Typ IgM oder IgG sowie urtikarielles Exanthem, Fieber, neutrophile Dermatitis, Knochenveränderungen und systemische serologische Inflammationszeichen [ 2 ]. Weiterhin sind z.…”