Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients

Abstract

Background: Schnitzler syndrome is characterized by an urticarial rash, a monoclonal gammopathy, and clinical, histological, and biological signs of neutrophilmediated inflammation. The aim of this study was to assess the applicability and validity of the existing diagnostic criteria in real-life patients. Methods: This multicentric study was conducted between 2009 and 2014 in 14 hospitals in which patients with Schnitzler syndrome or controls with related disorders were followed up. We compared the sensitivities and specificities and calculated the positive and negative predictive values of the Lipsker and of the Strasbourg criteria for the patients with Schnitzler syndrome and for the controls. We included 42 patients with Schnitzler syndrome, 12 with adult-onset Still's disease, 7 with cryopyrin-associated periodic disease, 9 with Waldenstr€ om disease, and 10 with chronic spontaneous urticaria. Results: All patients with Schnitzler syndrome met the Lipsker criteria. According to the Strasbourg criteria, 34 patients had definite Schnitzler syndrome, five had probable Schnitzler syndrome, and three did not meet the criteria. One control met the Lipsker criteria and had probable Schnitzler syndrome according to the Strasbourg criteria. Sensitivity and specificity of the Lipsker criteria were 100% and 97%, respectively. For the Strasbourg criteria, sensitivity for definite and probable diagnosis was 81% and 93%, respectively, with a corresponding specificity of 100% and 97%. Conclusion: Diagnostic criteria currently in use to diagnose Schnitzler syndrome are reliable. More investigations must be done to attest their efficiency in patients with recent-onset manifestations.

Neutrophilic urticarial dermatosis: an entity bridging monogenic and polygenic autoinflammatory disorders, and beyond

“…Since its original description, NUD has been accepted as an autonomous entity. Numerous new cases have been reported, and associated diseases have been confirmed and expanded . Table summarizes the associated diseases that have been published since our original publication in 2009.…”

Aortitis: A new feature of Schnitzler syndrome

“…Diagnosis relies on the validated criteria established by Lipsker et al 1 or the newer Strasbourg criteria 2, 3. We report a typical case of Schnitzler syndrome with a so-far not-described feature: diffuse aortitis.…”

Urticarial Syndromes

“…Schnizler syndrome is characterized by an urticarial rash and monoclonal gammopathy clinically and neutrophil-mediated inlammation histologically [57]. An urticarial rash and usually IgM but rarely IgG monoclonal gammopathy are present with a chronic patern in all the patients.…”

“…Recurrent fever, bone or joint pain, increased bone density, hepato-or splenomegaly, lymphadenopathy, and elevated acute-phase reactants are also accepted as minor criteria [58]. Approximately, 300 cases have been identiied in the literature [57]. Risk of developing a lymphoproliferative disorder at an approximate rate of 15% has been reported in the 10-year follow-up, although the syndrome usually has a benign course.…”