de Koning Heleen D, Bodar Evelien J, van der Meer Jos W M, Simon Anna
Division of General Internal Medicine, Department of Internal Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
Semin Arthritis Rheum. 2007 Dec;37(3):137-48. doi: 10.1016/j.semarthrit.2007.04.001. Epub 2007 Jun 21.
Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy, accompanied by intermittent fever, arthralgia or arthritis, bone pain, and lymphadenopathy. Our objectives are to systematically review disease characteristics of Schnitzler syndrome and collect follow-up information to gain insight into treatment efficacy and long-term prognosis.
PubMed and MEDLINE databases (1966-2006) were searched, using the key words "Schnitzler syndrome," and the combination of "urticaria" with "monoclonal gammopathy," "immunoglobulin M (IgM)," or "paraproteinemia," as well as secondary references. Data on a total of 94 patients who met the criteria for Schnitzler syndrome were reviewed. Questionnaires sent to all authors retrieved additional follow-up data on 43 patients, resulting in a mean follow-up of 9.5 years after onset of symptoms, and a follow-up of 20 years or more in 10 patients.
Symptoms, signs, and laboratory findings as found in the 94 patients are reviewed in detail. There have been promising developments in therapeutic options, especially antiinterleukin-1 treatment, which induced complete remission in all 8 patients treated so far. To date, no spontaneous complete remissions have been reported. Patients with Schnitzler syndrome showed no increased mortality during the present follow-up. However, they had a 10-year risk of 15% of developing a lymphoproliferative disorder, most notably Waldenström's macroglobulinemia. Three cases of type amyloid A (AA) amyloidosis associated with Schnitzler syndrome were reported.
Schnitzler syndrome is a disabling disorder which affects multiple systems and which can be considered as an autoinflammatory syndrome. There are new, effective treatment options, but close monitoring remains warranted because of the increased risk of lymphoproliferative disease.
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，伴有间歇性发热、关节痛或关节炎、骨痛及淋巴结病。我们的目的是系统回顾施尼茨勒综合征的疾病特征并收集随访信息，以深入了解治疗效果和长期预后。
检索了PubMed和MEDLINE数据库（1966年至2006年），使用关键词“施尼茨勒综合征”，以及“荨麻疹”与“单克隆丙种球蛋白病”、“免疫球蛋白M（IgM）”或“副蛋白血症”的组合，还有二次参考文献。对总共94例符合施尼茨勒综合征标准的患者的数据进行了回顾。向所有作者发送问卷，获取了另外43例患者的随访数据，症状出现后的平均随访时间为9.5年，10例患者的随访时间为20年或更长。
详细回顾了94例患者的症状、体征和实验室检查结果。治疗选择方面有了令人鼓舞的进展，尤其是抗白细胞介素-1治疗，在目前已治疗的所有8例患者中均诱导了完全缓解。迄今为止，尚未报告有自发完全缓解的情况。在本次随访期间，施尼茨勒综合征患者的死亡率没有增加。然而，他们有15%的10年风险发展为淋巴增殖性疾病，最常见的是华氏巨球蛋白血症。报告了3例与施尼茨勒综合征相关的A型淀粉样变性（AA）病例。
施尼茨勒综合征是一种影响多个系统的致残性疾病，可被视为一种自身炎症综合征。有新的有效治疗选择，但由于淋巴增殖性疾病风险增加，仍需密切监测。
