TY - JOUR
T1 - Schnitzler's syndrome
T2 - Diagnosis, treatment, and follow-up
AU - Simon, A.
AU - Asli, B.
AU - Braun-Falco, M.
AU - De Koning, H.
AU - Fermand, J. P.
AU - Grattan, C.
AU - Krause, K.
AU - Lachmann, H.
AU - Lenormand, C.
AU - Martinez-Taboada, V.
AU - Maurer, M.
AU - Peters, M.
AU - Rizzi, R.
AU - Rongioletti, F.
AU - Ruzicka, T.
AU - Schnitzler, L.
AU - Schubert, B.
AU - Sibilia, J.
AU - Lipsker, D.
PY - 2013/5
Y1 - 2013/5
N2 - 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.
AB - 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.
KW - anakinra
KW - monoclonal gammopathy
KW - neutrophilic urticarial dermatosis
KW - Schnitzler's syndrome
KW - urticaria
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U2 - 10.1111/all.12129
DO - 10.1111/all.12129
M3 - Article
C2 - 23480774
AN - SCOPUS:84876685876
VL - 68
SP - 562
EP - 568
JO - Allergy: European Journal of Allergy and Clinical Immunology
JF - Allergy: European Journal of Allergy and Clinical Immunology
SN - 0105-4538
IS - 5
ER -