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Schnitzler's syndrome: diagnosis, treatment, and follow-up.

Authors :
Simon, A.
Asli, B.
Braun‐Falco, M.
Koning, H.
Fermand, J.‐P.
Grattan, C.
Krause, K.
Lachmann, H.
Lenormand, C.
Martinez‐Taboada, V.
Maurer, M.
Peters, M.
Rizzi, R.
Rongioletti, F.
Ruzicka, T.
Schnitzler, L.
Schubert, B.
Sibilia, J.
Lipsker, D.
Source :
Allergy. May2013, Vol. 68 Issue 5, p562-568. 7p. 1 Diagram, 4 Charts.
Publication Year :
2013

Abstract

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. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
01054538
Volume :
68
Issue :
5
Database :
Academic Search Index
Journal :
Allergy
Publication Type :
Academic Journal
Accession number :
87016256
Full Text :
https://doi.org/10.1111/all.12129