Edited by: Thomas Bieber
Schnitzler's syndrome: diagnosis, treatment, and follow-up
Article first published online: 9 MAR 2013
© 2013 John Wiley & Sons A/S. Published by Blackwell Publishing Ltd
Volume 68, Issue 5, pages 562–568, May 2013
How to Cite
Schnitzler's syndrome: diagnosis, treatment, and follow-up. Allergy 2013;68: 562−568 DOI: 10.1111/all.12129., , , , , , , , , , , , , , , , , , .
- Issue published online: 17 APR 2013
- Article first published online: 9 MAR 2013
- Manuscript Accepted: 5 JAN 2013
- monoclonal gammopathy;
- neutrophilic urticarial dermatosis;
- Schnitzler's syndrome;
Schnitzler's syndrome is characterized by recurrent urticarial rash and monoclonal gammopathy, associated with clinical and biological signs of inflammation and a long-term risk of AA amyloidosis and overt lymphoproliferation. An extensive literature review was performed, and the following questions were addressed during an expert meeting: In whom should Schnitzler's syndrome be suspected? How should the diagnosis of Schnitzler's syndrome be established? How should a patient with Schnitzler's syndrome be treated? How should a patient with Schnitzler's syndrome be followed up?. A diagnosis of Schnitzler's syndrome is considered definite in any patient with two obligate criteria: a recurrent urticarial rash and a monoclonal IgM gammopathy, and two of the following minor criteria: recurrent fever, objective signs of abnormal bone remodeling, elevated CRP level or leukocytosis, and a neutrophilic infiltrate on skin biopsy. It is considered probable, if only 1 minor criterion is present. In patients with monoclonal IgG gammopathies, diagnosis is definite if three minor criteria are present and possible if two are present. First-line treatment in patients with significant alteration of quality of life or persistent elevation of markers of inflammation should be anakinra. Follow-up should include clinical evaluation, CBC and CRP every 3 months and MGUS as usually recommended.