肉芽肿性多血管炎垂体受累临床分析并文献复习  

The clinical analysis of patients of granulomatosis with polyangiitis accompanied by pituitary involvement and literature review

作　　者：王艳妮[1] 杨金奎[1,2] 高圆[1] 王振刚[1] 崔莉[1] 陈楠[1] 周建博[2] WANG Yan-ni;YANG Jin-kui;GAO Yuan(Department of Rheumatology,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China)

机构地区：[1]首都医科大学附属北京同仁医院风湿科,北京100730 [2]首都医科大学附属北京同仁医院内分泌科,北京100730

出　　处：《中日友好医院学报》2018年第6期347-351,F0002,共6页Journal of China-Japan Friendship Hospital

基　　金：首都医科大学附属北京同仁医院院内种子基金

摘　　要：目的:初步探讨肉芽肿性多血管炎(GPA)垂体受累的临床特征、影像学特点、治疗及预后。方法:收集本中心住院诊为GPA垂体受累的病例5例,分析其临床资料。对本中心及相关文献报告中共72例GPA垂体受累患者的临床特征进行分析。结果:本中心5例GPA垂体受累患者均有耳鼻喉受累、均出现中枢性尿崩症及垂体MRI异常,均应用激素及环磷酰胺治疗,5例系统受累均缓解;4例尿崩症缓解,1例尿崩症未缓解。结合文献数据,GPA垂体受累中65.3%为局限型GPA,最常见表现为中枢性尿崩症(88.9%)、最常累及耳鼻喉(72.2%)。MRI检查发现垂体异常94.4%,常见表现为垂体增大、垂体柄异常、不均匀强化及垂体后叶高信号影消失。患者接受激素和/或免疫抑制剂治疗,系统受累表现均改善,55.6%垂体功能紊乱未得到完全纠正。结论:GPA垂体受累多见于局限型GPA患者,常伴耳鼻喉受累。尿崩症是最常见的临床表现,MRI有助于发现垂体异常。GPA累及垂体罕见,早期诊断早期治疗可能有助于逆转垂体功能紊乱。Objective:To study the clinical characteristics,imaging features,treatment and prognosis of granulomatosis with polyangiitis(GPA)accompanied by pituitary involvement.Methods:Five GPA cases with pituitary involvement in our department formed the basis of the study.The clinical features of 72 GPA cases(including5 cases in our department)with pituitary involvement were analyzed and relevant literature was reviewed.Results:All the 5 patients in our department had Ear-nose-throat(ENT)involvement,central diabetes insipidus and abnormal pituitary imaging.All were managed with corticosteroids in combination with cyclophosphamide.After treatment,GPA was in systemic remission in all the 5 patients,4 patients recovered from pituitary dysfunction but 1 patient had persistent central diabetes insipidus and was still under hormone replacement therapy.Among the 72 GPA cases,65.3% existed in the limited form of GPA.ENT involvement seen in 72.2% and central diabetes insipidus seen in 88.9%.94.4% had abnormal pituitary imaging.The common MRI findings were enlargement of pituitary gland,involved stalk,heterogeneous enhancement and loss of posterior pituitary signal.Despite a high rate of systemic disease improvement,55.6% of the patients had persistent pituitary dysfunction.Conclusion:Most of the GPA patients with pituitary dysfunction existed in the limited form of GPA.ENT involvement was the most common type of systemic involvement.Central diabetes insipidus was the predominant manifestation of pituitary disease.Pituitary involvement in GPA is rare,it is important to recognize and treat these patients early to minimize the risk of irreversible pituitary function loss.

关 键 词：肉芽肿性多血管炎 中枢性尿崩症 垂体 

分 类 号：R593.2[医药卫生—内科学]