title: Schnitzler's syndrome: diagnosis, treatment, and follow-up.
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.
authors.affiliation: Department of General Internal Medicine/Infectious Diseases, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands.
authors.forename: A
authors.initials: A
authors.lastname: Simon
authors.forename: B
authors.initials: B
authors.lastname: Asli
authors.forename: M
authors.initials: M
authors.lastname: Braun-Falco
authors.forename: H
authors.initials: H
authors.lastname: De Koning
authors.forename: J-P
authors.initials: JP
authors.lastname: Fermand
authors.forename: C
authors.initials: C
authors.lastname: Grattan
authors.forename: K
authors.initials: K
authors.lastname: Krause
authors.forename: H
authors.initials: H
authors.lastname: Lachmann
authors.forename: C
authors.initials: C
authors.lastname: Lenormand
authors.forename: V
authors.initials: V
authors.lastname: Martinez-Taboada
authors.forename: M
authors.initials: M
authors.lastname: Maurer
authors.forename: M
authors.initials: M
authors.lastname: Peters
authors.forename: R
authors.initials: R
authors.lastname: Rizzi
authors.forename: F
authors.initials: F
authors.lastname: Rongioletti
authors.forename: T
authors.initials: T
authors.lastname: Ruzicka
authors.forename: L
authors.initials: L
authors.lastname: Schnitzler
authors.forename: B
authors.initials: B
authors.lastname: Schubert
authors.forename: J
authors.initials: J
authors.lastname: Sibilia
authors.forename: D
authors.initials: D
authors.lastname: Lipsker
mesh_terms: D005500:Follow-Up Studies; D006801:Humans; D019873:Schnitzler Syndrome / Q000175:diagnosis* / Q000188:drug therapy*