Abstract：
Schnitzler\'s Syndrome (SS) is a rare late-onset acquired autoinflammatory disorder which consists of chronic urticaria associated with a monoclonal IgM-kappa gammopathy, arthralgias, skeletal hyperostosis, lymphadenopathy, and recurrent constitutional symptoms. The average age of diagnosis is 51 years with a slight male predominance with a male to female ratio of 1.6. Diagnosis of SS requires the presence of 2 major criteria including chronic urticaria and monoclonal IgM along with at least two of the following minor criteria: recurrent intermittent fevers, bone pain, arthralgias, elevated erythrocyte sedimentation rate (ESR), neutrophilic dermal infiltrate on skin biopsy, and leukocytosis or elevated C-reactive protein (CRP). Early diagnosis and clinical awareness are paramount in SS as it is associated with a 15-20% risk of lymphoproliferative malignancy. The median overall survival is 12.8 years. We present a case of a 39-year-old female with new onset urticaria associated with recurrent fevers and joint pain. Symptoms were refractory to steroids, and high dose antihistamines. Multi-disciplinary evaluation resulted in the ultimate diagnosis of Schnitzler\'s Syndrome. The patient was ultimately treated with canakinumab (Il-1 inhibitor), with near resolution of symptoms. This case demonstrates the importance of a broad differential diagnosis and maintaining a high clinical suspicion for rare diseases when presented with a complex form of an otherwise common condition.
摘要：
Schnitzler综合征（SS）是一种罕见的迟发性获得性自身炎症性疾病，由与单克隆IgM-κ丙种球蛋白病相关的慢性荨麻疹组成。关节痛,骨骼肥大，淋巴结病，和反复发作的全身症状。诊断的平均年龄为51岁，男性占主导地位，男女比例为1.6。SS的诊断需要存在两个主要标准，包括慢性荨麻疹和单克隆IgM以及至少两个以下次要标准：复发性间歇性发烧，骨痛,关节痛,红细胞沉降率（ESR）升高，皮肤活检时嗜中性皮肤浸润，和白细胞增多或C反应蛋白（CRP）升高。在SS中，早期诊断和临床意识至关重要，因为它与淋巴增生性恶性肿瘤的15-20％风险相关。中位总生存期为12.8年。我们介绍了一例39岁女性，患有新发荨麻疹，并伴有反复发烧和关节痛。症状是类固醇难治的，和高剂量抗组胺药.多学科评估可最终诊断Schnitzler综合征。患者最终用canakinumab（IL-1抑制剂）治疗，症状接近消退。此病例证明了广泛鉴别诊断的重要性，并在出现复杂形式的其他常见疾病时保持对罕见疾病的高度临床怀疑。
