Bashir Maaman, Bettendorf Brittany, Hariman Richard
Division of Rheumatology, Department of Medicine, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
Division of Rheumatology, Department of Medicine, University of Iowa, Iowa City, IA 52242, USA.
Case Rep Rheumatol. 2018 Mar 8;2018:7041576. doi: 10.1155/2018/7041576. eCollection 2018.
Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy (IgM in more than 90% of the cases). It is difficult to distinguish from other neutrophilic urticarial dermatoses, and diagnosis is based on the Strasbourg criteria. Interleukin-1 is considered the key mediator, and interleukin-1 inhibitors are considered first line treatment. Here, we present two cases of Schnitzler syndrome, both successfully treated with anakinra.
To increase awareness regarding clinical presentation, diagnosis, and treatment of this rare disorder.
We describe the clinical features and disease course of two patients with Schnitzler syndrome, diagnosed using the Strasbourg criteria. Both were treated with anakinra with remarkable response to therapy.
Schnitzler syndrome is a rare and underdiagnosed disorder. High suspicion should be maintained in patients with chronic urticaria-like dermatoses, intermittent fevers, and arthralgias. A serum protein electrophoresis and immunofixation should be performed in these patients. The diagnosis is important to recognize as Schnitzler syndrome is associated with malignancy. A lymphoproliferative disorder develops in about 20% of patients at an average of 7.6 years after onset of symptoms. Thus, patients warrant long-term follow-up. IL-1 inhibitors are extremely effective in relieving symptoms and are considered first line therapy.
施尼茨勒综合征是一种罕见疾病，其特征为慢性荨麻疹样皮疹和单克隆丙种球蛋白病（超过90%的病例为IgM）。它很难与其他嗜中性荨麻疹性皮肤病相区分，诊断基于斯特拉斯堡标准。白细胞介素-1被认为是关键介质，白细胞介素-1抑制剂被视为一线治疗药物。在此，我们报告两例施尼茨勒综合征病例，均用阿那白滞素成功治疗。
提高对这种罕见疾病临床表现、诊断和治疗的认识。
我们描述了两名符合斯特拉斯堡标准诊断的施尼茨勒综合征患者的临床特征和病程。两人均接受阿那白滞素治疗，对治疗反应显著。
施尼茨勒综合征是一种罕见且诊断不足的疾病。对于患有慢性荨麻疹样皮肤病、间歇性发热和关节痛的患者应高度怀疑。这些患者应进行血清蛋白电泳和免疫固定电泳检查。认识到施尼茨勒综合征与恶性肿瘤有关，诊断很重要。约20%的患者在症状出现后平均7.6年发生淋巴增殖性疾病。因此，患者需要长期随访。白细胞介素-1抑制剂在缓解症状方面极其有效，被视为一线治疗药物。
