韦格纳肉芽肿伴中枢性尿崩症并文献复习

A Case of Wegener’s Granulomatosis with Central Diabetes Insipidus:Report and Literature Review

目的:总结韦格纳肉芽肿(WG)伴垂体受累的临床特点和治疗策略。方法:通过1例WG伴中枢性尿崩症患者的临床诊治经过进行文献复习。结果:患者27岁女性,以咽痛伴听力下降为首发症状,1月后出现多饮、多尿,经病理活检及血清PR3-ANCA(+)确诊为不完全型WG,合并中枢性尿崩症。经糖皮质激素和免疫抑制剂治疗有效。结论:耳聋是WG少见临床表现,中枢性尿崩症更为罕见,垂体MRI是判定垂体病变性质并监测垂体病情的重要手段;环磷酰胺联合激素治疗有效。

Objective：To investigate the clinic feature and treatment of Wegener’s granulomatosis（WG）with pituitary gland injury.Methods：A case of WG with central diabetes insipidus was reported and literature was reviewed.Results：A 27-year-old female presented with sore throat,hearing loss,polydipsia and polyuria was diagnosed with WG with diabetes insipidus based on pathology and seropositive for PR3ANCA.Her clinical symptoms was improved after being treated with glucocorticoid and immunosuppressive drugs.Conclusion：Hearing loss is uncommon manifeatation,central diabetes insipidus in WG is rare.MRI of pituitary is the important method for deciding the kind of pituitary gland involvement and monitoring the development.The drugs of cyclophosphamide and glucocorticoid is effective.