Schnitzler's syndrome: Diagnosis, treatment, and follow-up

A. Simon, B. Asli, M. Braun-Falco, H. De Koning, J. P. Fermand, C. Grattan, K. Krause, H. Lachmann, C. Lenormand, V. Martinez-Taboada, M. Maurer, M. Peters, R. Rizzi, F. Rongioletti, T. Ruzicka, L. Schnitzler, B. Schubert, J. Sibilia, D. Lipsker

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)562-568
Number of pages7
JournalAllergy: European Journal of Allergy and Clinical Immunology
Volume68
Issue number5
DOIs
Publication statusPublished - May 2013

Keywords

  • anakinra
  • monoclonal gammopathy
  • neutrophilic urticarial dermatosis
  • Schnitzler's syndrome
  • urticaria

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

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