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Schnitzler's syndrome: diagnosis, treatment, and follow-up
- Source :
- Allergy; 562; 8; 0105-4538; 5; 68; ~Allergy~562~8~~~0105-4538~5~68~~
- Publication Year :
- 2013
-
Abstract
- Item does not contain fulltext<br />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.
Details
- Database :
- OAIster
- Journal :
- Allergy; 562; 8; 0105-4538; 5; 68; ~Allergy~562~8~~~0105-4538~5~68~~
- Publication Type :
- Electronic Resource
- Accession number :
- edsoai.on1284050323
- Document Type :
- Electronic Resource