阿那白滞素和沙利度胺对施尼茨勒综合征有有益反应。
Beneficial response to anakinra and thalidomide in Schnitzler's syndrome.
作者信息
de Koning H D, Bodar E J, Simon A, van der Hilst J C H, Netea M G, van der Meer J W M
机构信息
Radboud University Nijmegen Medical Centre, Department of General Internal Medicine, 6500 HB Nijmegen, The Netherlands.
出版信息
Ann Rheum Dis. 2006 Apr;65(4):542-4. doi: 10.1136/ard.2005.045245. Epub 2005 Aug 11.
BACKGROUND
Schnitzler's syndrome is an inflammatory disorder characterised by chronic urticarial rash and monoclonal gammopathy, accompanied by periodic fever, arthralgia or arthritis, and bone pain. The cause and treatment are still unknown.
OBJECTIVE
To assess treatment with thalidomide and an interleukin 1 receptor antagonist, anakinra, in Schnitzler's syndrome.
CASE REPORTS
Three patients with Schnitzler's syndrome are described, one with IgM gammopathy, two with IgG type. In one patient, thalidomide induced complete remission, but was stopped because of polyneuropathy. Anakinra 100 mg daily in all three patients led to disappearance of fever and skin lesions within 24 hours. After a follow up of 6-18 months, all patients are free of symptoms.
CONCLUSION
Anakinra proved to be effective in three patients with Schnitzler's syndrome. This treatment is preferable to thalidomide, which induced a complete remission in one of our patients, as it has fewer side effects.
背景
施尼茨勒综合征是一种炎症性疾病，其特征为慢性荨麻疹皮疹和单克隆丙种球蛋白病，伴有周期性发热、关节痛或关节炎以及骨痛。病因和治疗方法仍不明确。
目的
评估沙利度胺和白细胞介素1受体拮抗剂阿那白滞素治疗施尼茨勒综合征的效果。
病例报告
描述了3例施尼茨勒综合征患者，1例为IgM型丙种球蛋白病，2例为IgG型。1例患者使用沙利度胺后完全缓解，但因多发性神经病停药。所有3例患者每日使用100mg阿那白滞素，24小时内发热和皮肤病变消失。随访6 - 18个月后，所有患者均无症状。
结论
阿那白滞素被证明对3例施尼茨勒综合征患者有效。这种治疗方法优于沙利度胺（我们的1例患者使用沙利度胺后完全缓解），因为它的副作用更少。
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