Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
Unité d'Immuno-Hématologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France.
Allergy. 2017 Feb;72(2):177-182. doi: 10.1111/all.13035. Epub 2016 Sep 12.
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.
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.
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%.
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.
Schnitzler 综合征的特征为荨麻疹样皮疹、单克隆丙种球蛋白病和中性粒细胞介导的炎症的临床、组织学和生物学征象。本研究旨在评估现有诊断标准在真实患者中的适用性和有效性。
本多中心研究于 2009 年至 2014 年在 14 家医院进行，其中 Schnitzler 综合征患者或具有相关疾病的对照患者接受了随访。我们比较了 Lipsker 和 Strasbourg 标准对 Schnitzler 综合征患者和对照患者的敏感性和特异性，并计算了阳性和阴性预测值。我们纳入了 42 例 Schnitzler 综合征患者、12 例成人Still 病患者、7 例 cryopyrin 相关周期性疾病患者、9 例 Waldenström 病患者和 10 例慢性自发性荨麻疹患者。
所有 Schnitzler 综合征患者均符合 Lipsker 标准。根据 Strasbourg 标准，34 例患者患有明确的 Schnitzler 综合征，5 例患者患有可能的 Schnitzler 综合征，3 例患者不符合标准。1 例对照患者符合 Lipsker 标准，根据 Strasbourg 标准患有可能的 Schnitzler 综合征。Lipsker 标准的敏感性和特异性分别为 100%和 97%。对于 Strasbourg 标准，明确和可能诊断的敏感性分别为 81%和 93%，相应的特异性分别为 100%和 97%。
目前用于诊断 Schnitzler 综合征的诊断标准是可靠的。必须进行更多的研究以证明其在近期发病患者中的有效性。
