Department of Environmental Dermatology and Allergy, Medical University of Graz, Graz, Austria.
Int J Dermatol. 2009 Nov;48(11):1190-4. doi: 10.1111/j.1365-4632.2009.04151.x.
Schnitzler syndrome is a rare disease characterized by a chronic urticarial eruption and monoclonal gammopathy, as well as clinical and laboratory signs of inflammation. The pathophysiology is still unknown, although various autoantibody-mediated mechanisms have been described. Complete remission of symptoms has been reported recently in patients with Schnitzler syndrome treated with anakinra, an interleukin-1 receptor antagonist.
Two patients with Schnitzler syndrome treated with anakinra therapy are presented.
We report two cases of nearly complete remission of symptoms in Schnitzler syndrome after the initiation of anakinra therapy, and the first observation of a relapse under continuous daily anakinra therapy. A review of the published literature on the treatment of Schnitzler syndrome with anakinra is presented.
Based on published data, monotherapy with anakinra is currently the most promising treatment for Schnitzler syndrome, because it is able to induce complete remission of symptoms.
Schnitzler 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，以及炎症的临床和实验室征象。尽管已经描述了各种自身抗体介导的机制，但病理生理学仍不清楚。最近有报道称，使用白细胞介素-1 受体拮抗剂 anakinra 治疗 Schnitzler 综合征患者可使症状完全缓解。
介绍了两名接受 anakinra 治疗的 Schnitzler 综合征患者。
我们报告了两例 Schnitzler 综合征患者在开始 anakinra 治疗后症状几乎完全缓解，并且首次观察到在连续每日 anakinra 治疗下复发。对 anakinra 治疗 Schnitzler 综合征的已发表文献进行了回顾。
基于已发表的数据，anakinra 单药治疗目前是 Schnitzler 综合征最有前途的治疗方法，因为它能够诱导症状完全缓解。
