L Gusdorf1, B Asli2, S Barbarot3, A Néel4, A Masseau4, X Puéchal5, J-E Gottenberg6, G Grateau7, C Blanchard-Delaunay8, R Rizzi9, F Lifermann10, X Kyndt11, F Aubin12, D Bessis13, T Boye14, S Gayet15, F Rongioletti16, E Sauleau17, J-P Fermand2, D Lipsker1. 1. Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg, Strasbourg, France. 2. Unité d'Immuno-Hématologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France. 3. Service de Dermatologie, CHU Hôtel-Dieu, Nantes, France. 4. Service de Médecine Interne, CHU Hôtel Dieu, Nantes, France. 5. Service de Médecine Interne, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France. 6. Service de Rhumatologie, Hôpitaux Universitaires de Strasbourg, Strasbourg, France. 7. Service de Médecine Interne, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris, Paris, France. 8. Service de Médecine Interne, Centre Hospitalier, Niort, France. 9. Department of Emergency and Organ Transplantation, Section of Hematology with Transplantation, University of Bari, Bari, Italy. 10. Service de Médecine Interne, Centre Hospitalier, Dax, France. 11. Service de Médecine Interne, Centre Hospitalier, Valenciennes, France. 12. Service de Dermatologie, CHU de Besançon, Besançon, France. 13. Service de Dermatologie, CHU Saint-Eloi, Montpellier, France. 14. Service de Dermatologie, Hôpital d'Instruction des Armées Sainte-Anne, Toulon, France. 15. Service de Médecine Interne, Gériatrie et Thérapeutique, CHU de la Timone, Marseille, France. 16. Department of Dermatology, University of Genova, Genova, Italy. 17. Service de Santé Publique, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
Abstract
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 Waldenström 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.
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 Waldenström 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.
Authors: Hideyuki Ujiie; David Rosmarin; Michael P Schön; Sonja Ständer; Katharina Boch; Martin Metz; Marcus Maurer; Diamant Thaci; Enno Schmidt; Connor Cole; Kyle T Amber; Dario Didona; Michael Hertl; Andreas Recke; Hanna Graßhoff; Alexander Hackel; Anja Schumann; Gabriela Riemekasten; Katja Bieber; Gant Sprow; Joshua Dan; Detlef Zillikens; Tanya Sezin; Angela M Christiano; Kerstin Wolk; Robert Sabat; Khalaf Kridin; Victoria P Werth; Ralf J Ludwig Journal: Front Med (Lausanne) Date: 2022-06-09
Authors: Carlo Basile; Luigi Rossi; Francesco Casucci; Annalisa Teutonico; Pasquale Libutti; Piero Lisi; Carlo Lomonte; Raffaele Manna Journal: Clin Kidney J Date: 2017-07-26