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

被引：66

作者：

Gusdorf, L. ^{[1
]}

Asli, B. ^{[2
]}

Barbarot, S. ^{[3
]}

Neel, A. ^{[4
]}

Masseau, A. ^{[4
]}

Puechal, X. ^{[5
]}

Gottenberg, J-E. ^{[6
]}

Grateau, G. ^{[7
]}

Blanchard-Delaunay, C. ^{[8
]}

Rizzi, R. ^{[9
]}

Lifermann, F. ^{[10
]}

Kyndt, X. ^{[11
]}

Aubin, F. ^{[12
]}

Bessis, D. ^{[13
]}

Boye, T. ^{[14
]}

Gayet, S. ^{[15
]}

Rongioletti, F. ^{[16
]}

Sauleau, E. ^{[17
]}

Fermand, J-P. ^{[2
]}

Lipsker, D. ^{[1
]}

机构：

[1] Hop Univ Strasbourg, Dermatol Clin, Strasbourg, France

[2] Hop St Louis, Assistance Publ Hop Paris, Unite Immunohematol, Paris, France

[3] CHU Hotel Dieu, Serv Dermatol, Nantes, France

[4] CHU Hotel Dieu, Serv Med Interne, Nantes, France

[5] Hop Cochin, Assistance Publ Hop Paris, Serv Med Interne, Paris, France

[6] Hop Univ Strasbourg, Serv Rhumatol, Strasbourg, France

[7] Hop Tenon, Assistance Publ Hop Paris, Serv Med Interne, Paris, France

[8] Ctr Hosp, Serv Med Interne, Niort, France

[9] Univ Bari, Sect Hematol Transplantat, Dept Emergency & Organ Transplantat, Bari, Italy

[10] Ctr Hosp, Serv Med Interne, Dax, France

[11] Ctr Hosp, Serv Med Interne, Valenciennes, France

[12] CHU Besancon, Serv Dermatol, Besancon, France

[13] CHU St Eloi, Serv Dermatol, Montpellier, France

[14] Hop Instruct Armees St Anne, Serv Dermatol, Toulon, France

[15] CHU Timone, Serv Med Interne Genatrie & Therapeut, Marseille, France

[16] Univ Genoa, Dept Dermatol, Genoa, Italy

[17] Hop Univ Strasbourg, Serv Sante Publ, Strasbourg, France

来源：

ALLERGY | 2017年 / 72卷 / 02期

关键词：

auto-inflammatory disease; diagnostic criteria; monoclonal gammopathy; neutrophilic urticarial dermatosis; Schnitzler syndrome; CHRONIC URTICARIA; FOLLOW-UP; VARIANT; GAMMOPATHY;

D O I：

10.1111/all.13035

中图分类号：

R392 [医学免疫学];

学科分类号：

100102 ;

摘要：

Background: Schnitzler syndrome is characterized by an urticarial rash, a monoclonal gammopathy, and clinical, histological, and biological signs of neutrophil-mediated 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 Waldenstrom 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.

引用

页码：177 / 182

页数：6

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