Publications

Schnitzler’s syndrome is characterized by recurrent urticarial rash and monoclo- nal gammopathy, associated with clinical and biological signs of inflammation and a long-term risk of AA amyloidosis and overt lymphoproliferation. An exten- sive 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 Schnit- zler’s syndrome be followed up?. A diagnosis of Schnitzler’s syndrome is consid- ered 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 prob- able, if only 1 minor criterion is present. In patients with monoclonal IgG gamm- opathies, diagnosis is definite if three minor criteria are present and possible if two are present. First-line treatment in patients with significant alteration of qual- ity 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.