Marazzi R, Pareyson D, Boiardi A, Corbo M, Scaioli V, Sghirlanzoni A
Department of Neurology, Istituto Neurologico C. Besta, Milan, Italy.
J Neurol. 1992 Jul;239(6):317-21. doi: 10.1007/BF00867587.
Peripheral neuropathy associated with bronchial asthma, multisystem organ dysfunction and idiopathic hypereosinophilia may be found in Churg-Strauss syndrome, hypereosinophilic syndrome and polyarteritis nodosa. Some authors have diagnosed their patients according to the presence in tissue biopsies of the three histological criteria of Churg and Strauss (necrotizing vasculitis, tissue eosinophilic infiltration, extravascular granulomas). We have observed three patients with a common history of a prodromal phase of allergic diseases (bronchial asthma and rhinitis) followed by a vasculitic phase with mononeuritis multiplex, purpura and arthritis, associated with hypereosinophilia of more than 1500 cells/mm3. All responded well to steroid treatment. Sural nerve biopsy revealed true vasculitis in two of these cases and a mild perivascular inflammatory infiltration in the other. On the basis of their characteristic clinical pattern, we think that our cases best fit the diagnosis of Churg-Strauss syndrome even though the typical histological features were not found in the sural nerves examined.
与支气管哮喘、多系统器官功能障碍和特发性嗜酸性粒细胞增多相关的周围神经病变可见于变应性肉芽肿性血管炎、嗜酸性粒细胞增多综合征和结节性多动脉炎。一些作者根据组织活检中是否存在Churg和Strauss的三项组织学标准(坏死性血管炎、组织嗜酸性粒细胞浸润、血管外肉芽肿)来诊断他们的患者。我们观察了三名患者,他们都有过敏性疾病(支气管哮喘和鼻炎)前驱期的共同病史,随后进入血管炎期,伴有多发性单神经炎、紫癜和关节炎,并伴有嗜酸性粒细胞增多超过1500个细胞/mm³。所有患者对类固醇治疗反应良好。在其中两例患者的腓肠神经活检显示为真性血管炎,另一例为轻度血管周围炎性浸润。基于其特征性的临床模式,我们认为我们的病例最符合变应性肉芽肿性血管炎的诊断,尽管在所检查的腓肠神经中未发现典型的组织学特征。