垂体及垂体柄病变.docVIP

垂体及垂体柄病变（漏斗垂体炎）与免疫球蛋白G4相关的全身系统性疾病具有相关性：一种新的临床病症Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: an emerging clinical entity. 出处：Endocr J. 2009; 56(9):1033-41 作者：Shimatsu A, Oki Y, Fujisawa I, Sano TPMID译者： F1000因子：6 评级：推荐 文章类型： 阅读方式： ?? ?垂体炎性病变比较罕见。目前，日本提出了免疫球蛋白G4(IgG4)相关性全身系统性疾病的理念，并报道了自2000年以来20多例垂体漏斗部的相关疾病。 ?? ?在此我们回顾了已发表过的病例报告及科学会议上的摘要报告。几乎所有的病例都是中老年男性表现出不同程度的垂体功能减退及糖尿病尿崩症症状，并伴有垂体柄增厚和/或垂体体积增大。垂体结构萎缩与糖皮质激素治疗有关，即使作为类似肾上腺皮质功能不全的替代物小剂量范围应用（也会影响垂体）。 ?? ?某些垂体前叶功能不全可通过对糖皮质激素的应用管理而改善。糖皮质激素治疗前出现IgG4相关性全身系统性疾病及血清IgG4浓度增高成为准确诊断IgG4相关性漏斗垂体炎的主要依据。推测自身免疫在IgG4相关性全身系统性疾病的发病机制中起作用但尚未证实。 ?? ?某些病例伴有肥厚性硬脑脊膜炎及副鼻窦炎提示鞍区及鞍旁结构均参与了该慢性炎症过程。因此我们将这一疾病归类为与IgG4相关性全身系统疾病相关的漏斗垂体炎的亚型，而不是其他类型的自身免疫性垂体疾病。 Inflammatory lesions of the pituitary gland are rarely encountered. Recently, the concept of immunoglobulin G4 (IgG4)-related systemic disease was proposed in Japan, and more than 20 cases have been reported as possibly associated with infundibulo-hypophysitis since 2000. We herein review such case reports in the published literature and in the abstracts of scientific meetings. Almost all cases involved middle-aged to elderly men presenting with various degrees of hypopituitarism and diabetes insipidus and demonstrating a thickened pituitary stalk and/or pituitary mass. These structures shrank remarkably in response to glucocorticoid therapy, even in a lower dose range similar to that prescribed as a replacement for adrenocortical insufficiency. Some of the anterior pituitary insufficiencies were also resolved by glucocorticoid administration. The presence of IgG4-related systemic disease and an elevated serum IgG4 level before glucocorticoid therapy were the main clues to a correct diagnosis of IgG4-related infundibulo-hypophysitis. Autoimmunity is suggested but not yet established to play a role in the pathogenesis for IgG4-related systemic disease. The fact that hypertrophic pachymeningitis and para-sinusitis ac