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[韦格纳肉芽肿病与中枢性尿崩症]

[Wegener's disease and central diabetes insipidus].

作者信息

Berthier S, Gil H, Bonneville J F, Magy N, Mallet H, Dupond J L

机构信息

Service de Médecine Interne, CHU J. Minjoz, Besançon.

出版信息

Ann Endocrinol (Paris). 2000 Dec;61(6):531-537.

Abstract

Vasculitis with central diabetes insipidus is a rare condition which must be recognized for proper management. We report a case of Wegener's granulomatosis with diabetes insipidus and review the literature concerning the clinical and radiological features of this association. We examined the data supporting the efficacy of cyclophosphamide in diabetes insipidus. Diabetes insipidus is inaugural in 46% of cases. Wegener's granulomatosis has no distinctive features when associated with diabetes insipidus. MR imaging reveals an enlargement of the posterior pituitary in 100% of cases. Cyclophosphamide is effective in 80% of cases. In Wegener's granulomatosis, pituitary vasculitis is a rare condition. MR imaging is the most accurate method for pituitary exploration. Cyclophosphamide is effective in the treatment of vasculitis lesions in Wegener's granulomatosis.

摘要

伴中枢性尿崩症的血管炎是一种罕见疾病,必须予以识别以便进行恰当治疗。我们报告一例伴有尿崩症的韦格纳肉芽肿病病例,并回顾有关这种关联的临床和放射学特征的文献。我们研究了支持环磷酰胺治疗尿崩症有效性的数据。46%的病例中尿崩症为首发症状。韦格纳肉芽肿病与尿崩症相关时无明显特征。磁共振成像显示100%的病例垂体后叶增大。80%的病例中环磷酰胺有效。在韦格纳肉芽肿病中,垂体血管炎是一种罕见情况。磁共振成像是垂体检查最准确的方法。环磷酰胺对治疗韦格纳肉芽肿病的血管炎病变有效。

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