变应性肉芽肿性血管炎14例临床表现及周围神经损害

Clinical Manifestations and Peripheral Neuropathy in 14 Patients with Allergic Granulomatous Vasculitis

目的分析变应性肉芽肿性血管炎(Churg-Strauss sydrome,CSS)的临床特点以及周围神经受损的表现,以提高诊治水平。方法收集自2005年1月~2016年12月在北京大学第三医院收治的14例CSS患者的临床资料,回顾性分析其临床表现及周围神经受损的情况。结果 14例CSS患者中男性10例(71.4%),年龄20~78岁(53.6±16.2);外周血嗜酸性粒细胞平均升高20.7%(10.3%~55%);呼吸系统是最常受累的器官(100%),哮喘9例(64.3%),肺部浸润12例(85.7%);6例(42.9%)存在周围神经受损,以四肢周围神经尤其是下肢神经轴索损害为主,同时累及运动与感觉神经;CSS起病初期误诊率高达64.3%,起病至确诊间隔平均15.9 m(1~72 m)。结论 CSS是一种误诊率较高的全身多系统受累的血管炎,存在嗜酸性粒细胞升高,且临床和电生理结果提示非对称性以轴索损害为主的周围神经病患者,需注意CSS的可能。

Objective Analyze the clinical characteristics of eosinophilic granulomatosis with polyangiitis（ ChurgStrauss syndrome,CSS） and peripheral neuropathy,and to improve the diagnosis and treatment of CSS. Methods Medical records from all patients diagnosed with CSS who had been treated at Peking University Third Hospital,China,between January 2005 and December 2016 were retrospectively reviewed to identify the clinical manifestations and the damage of peripheral nerve. Results 14 patients were diagnosed with CSS. 10（ 71. 4%） of them were males,and from 20 to 78（ 53. 6± 16. 2） years old. Peripheral blood eosinophils increased,and the mean of eosinophils was 20. 7%,（ 10. 3% - 55%）.The most frequently involved organ respiratory system（ 100%）,9 cases of asthma（ 64. 3%） and 12 cases of pulmonary invasion（ 85. 7%）. Peripheral nerve injuries occurred in 6 patients（ 42. 9%）,especially in the lower extremities,and both the motor and sensory nerves were involved. The misdiagnosis rate of CSS at the early onset was very high（ 64. 3%）,and mean time of the onset to diagnosis interval was 15. 9 months（ 1 - 72 months）. Conclusion CSS was a vasculitis involved multisystem,and with a high rate of misdiagnosis. The patients with elevated eosinophils and axonal impairment of peripheral neuropathy,should be paid attention to the possibility of CSS.