垂体及垂体柄病变[解读]

垂体及垂体柄病变[解读] 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 T PMID:19926920 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 accompanied some cases suggested that both sellar and parasellar structures are involved in the chronic inflammation. We therefore classify this disorder not as a variant form of primary autoimmune hypophysitis but as a secondary form of infundibulo-hypophysitis associated with IgG4-related systemic disease. This article reviews all data recently published about immunoglobulin G4 (IgG4)-related infundibulo-hypophisitis. Since 2000, more than 20 cases have been published. The presence of IgG4-related systemic disease and an elevated serum IgG4 level before glucocorticoid therapy were the main clues to correct diagnosis. Hypertrophic pachymeningitis and para-sinusitis accompanied some cases and therefore the authors classified these cases not as a variant form of primary autoimmune hypophisitis but as a secondary form of infundibulo-hypophisitis associated with IgG4-related systemic disease. Infundibulo-hypophisitis can be classified according to pituitary involvement in adenohypophisitis, infundibulo-neurohypophisitis and panhypophisitis. Lymphocitic hypophisitis is one cause of primary infundibulo-hypophisitis and is considered to be an autoimmune disease. Recently, pituitary involvement has been described as an extra-pancreatic manifestation of autoimmune pancreatitis IgG4-related, which is a systemic disease characterized by extensive infiltration of IgG4-positive plasma cells and T-lymphocytes into various organs, most commonly the pancreas, bile duct, gallbladder, salivary gland, retroperitonium, kidney, lung and prostate. The disease occurs predominantly in elder men, differently from hypophisitis lymphocitic in which young women are mainly affected, and responds well to glucocorticoid therapy. Serum IgG4 levels and immunostaining with anti-IgG4 antibody are useful for making a diagnosis. Various degrees of hypopituitarism and diabetes insipidus are reported and in MRI, stalk thickness and pituitary mass can be accompanied by pachymeningitis and para-sinusitis. Pathogenesis is unknown; however, an allergic reaction can be involved . In suspect cases, IgG4 measurement is a non-invasive test that could assist the diagnosis . Further studies are needed before the IgG4 assessment can replace invasive diagnostic methods, such as pituitary biopsy, for hypophisitis diagnosis. 专家评价: 本文回顾了最近发表的关于免疫球蛋白G4(IgG4)相关的漏斗垂体炎病所有资料。自2000年以来已报 道了20多例。糖皮质激素治疗前免疫球蛋白G4相关性全身系统性疾病的发生及血清IgG4水平升高是其 主要诊断依据。某些病例伴有肥厚性硬脑膜炎及副鼻窦炎，因此研究人员将这些病例归类为与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漏斗炎病。 根据垂体受累部位可将漏斗垂体炎分为腺垂体炎、漏斗神经垂体炎、周围垂体炎。 淋巴性垂体炎是漏 斗垂体炎的主要病因，而且认为是一种自身免疫性疾病。 目前，垂体病变认为是与IgG4相关的自身免疫性胰腺炎的胰管外表现，自身免疫性胰腺炎是一种全身 性疾病，其主要临床特点是IgG4阳性的浆细胞及T淋巴细胞广泛渗出并侵入多个器官，常见的有胰腺、 胆管、胆囊、唾液腺、腹膜后、肾脏、肺及前列腺。 这类疾病多发生于老年男性，不同于青年女性病变主要侵犯垂体淋巴液的特点，且糖皮质激素治疗效果好。血清IgG4浓度及抗IgG4抗体染色有助于诊断。据报道常伴有不同程度的垂体功能减退及尿崩症，磁共振显示垂体柄厚度增加及垂体体积增大可伴有硬脑膜炎和副鼻窦炎。 发病机制尚不清楚，但与变态反应有关。在可疑病例中无创性IgG4测量可辅助诊断。需进一步研究IgG4评估前替代垂体炎有创检查的方法，如垂体活检. Andrea Glezer and Marcello Bronstein University of Sao Paulo Medical School , Brazil Diabetes & Endocrinology 求助全文