Abstract：
BACKGROUND: Recurring therapy resistant hives, accompanied by IgM-gammopathy, fever and joint pain can indicate Schnitzler syndrome, a rare autoimmune disorder. There is currently no approved treatment, but complete remission of symptoms can be induced with IL-1 antagonists.
METHODS: A patient with a history of chronic urticaria presented frequently at the outpatient clinic with severe hives and was treated unsuccessfully with antihistamines and omalizumab. After several years, additional symptoms such as joint pain, recurrent fever, and IgM-gammopathy developed. After the diagnostic criteria for Schnitzler syndrome were met, treatment with anakinra was initiated and resulted in an improvement of the symptoms. Shortly after the first injection, the patient developed large and painful erythematous lesions at the injection sites, leading to discontinuation of treatment and a rapid recurrence of symptoms. Subsequently, treatment with a longer-acting IL-1 antagonist (canakinumab) was initiated, resulting in a complete remission of symptoms.
CONCLUSIONS: This case report demonstrates that patients with urticarial symptoms that are not relieved by typical treatments should prompt repeated reassessments of the diagnosis, even years later, because gammopathy and other diagnostic criteria for Schnitzler syndrome can occur with a delay.
METHODS: A patient with a history of chronic urticaria presented frequently at the outpatient clinic with severe hives and was treated unsuccessfully with antihistamines and omalizumab. After several years, additional symptoms such as joint pain, recurrent fever, and IgM-gammopathy developed. After the diagnostic criteria for Schnitzler syndrome were met, treatment with anakinra was initiated and resulted in an improvement of the symptoms. Shortly after the first injection, the patient developed large and painful erythematous lesions at the injection sites, leading to discontinuation of treatment and a rapid recurrence of symptoms. Subsequently, treatment with a longer-acting IL-1 antagonist (canakinumab) was initiated, resulting in a complete remission of symptoms.
CONCLUSIONS: This case report demonstrates that patients with urticarial symptoms that are not relieved by typical treatments should prompt repeated reassessments of the diagnosis, even years later, because gammopathy and other diagnostic criteria for Schnitzler syndrome can occur with a delay.
摘要：
背景：反复治疗耐药的荨麻疹，伴有IgM丙种球蛋白病，发烧和关节疼痛可以表明Schnitzler综合征，一种罕见的自身免疫性疾病.目前没有批准的治疗方法，但可以用IL-1拮抗剂诱导症状的完全缓解。
方法：一名有慢性荨麻疹病史的患者经常在门诊就诊，出现严重的荨麻疹，但抗组胺药和奥马珠单抗治疗失败。几年后,其他症状，如关节痛，反复发烧,和IgM丙种球蛋白病发展。在满足Schnitzler综合征的诊断标准后，开始使用anakinra进行治疗，并改善了症状。第一次注射后不久，患者在注射部位出现较大且疼痛的红斑病变，导致停止治疗和症状快速复发。随后，开始使用长效IL-1拮抗剂（canakinumab）治疗，导致症状完全缓解。
结论：该病例报告表明，典型治疗方法不能缓解的荨麻疹症状患者应反复重新评估诊断，甚至几年后，因为Schnitzler综合征的丙种球蛋白病和其他诊断标准可能会延迟发生。
方法：一名有慢性荨麻疹病史的患者经常在门诊就诊，出现严重的荨麻疹，但抗组胺药和奥马珠单抗治疗失败。几年后,其他症状，如关节痛，反复发烧,和IgM丙种球蛋白病发展。在满足Schnitzler综合征的诊断标准后，开始使用anakinra进行治疗，并改善了症状。第一次注射后不久，患者在注射部位出现较大且疼痛的红斑病变，导致停止治疗和症状快速复发。随后，开始使用长效IL-1拮抗剂（canakinumab）治疗，导致症状完全缓解。
结论：该病例报告表明，典型治疗方法不能缓解的荨麻疹症状患者应反复重新评估诊断，甚至几年后，因为Schnitzler综合征的丙种球蛋白病和其他诊断标准可能会延迟发生。
