Department of Endocrinology, King Edward Memorial Hospital, Mumbai, Maharashtra 400012, India.
Singapore Med J. 2009 Nov;50(11):1080-4.
Autoimmune hypophysitis (AH) is a rare primary autoimmune inflammatory disorder involving the pituitary gland.
A retrospective analysis of the clinical features and outcome of patients diagnosed with AH between 1988 and 2006, was carried out.
15 patients (14 females and one male) with AH were identified. Three patients presented in the peripartum period. Headache, vomiting and visual field defects, suggestive of an expanding sellar mass, were the most common presenting symptoms (67 percent). The most common deficient hormone was adrenocorticotropic hormone (ACTH) (67 percent), followed by thyroid stimulating hormone (53 percent) and gonadotropins (40 percent). Imaging revealed a definite, enhancing sellar mass in 87 percent of the patients and stalk thickening in 33 percent of the patients. Three patients underwent surgery. On serial monitoring, the sellar mass regressed or disappeared spontaneously without any immunosuppressive treatment in the other ten patients with a definite sellar mass.
We report a higher female to male ratio and a lower incidence of peripartum cases in our series. Symptoms of mass effect were the most common presentation, while ACTH was the most commonly-deficient hormone. Surgery was rarely needed, and most patients experienced a spontaneous resolution of the mass.
自身免疫性垂体炎(AH)是一种罕见的累及垂体的原发性自身免疫性炎症性疾病。
对 1988 年至 2006 年间诊断为 AH 的患者的临床特征和结局进行回顾性分析。
共发现 15 例(14 例女性和 1 例男性)AH 患者。3 例患者出现在围产期。头痛、呕吐和视野缺损,提示鞍内肿块扩大,是最常见的首发症状(67%)。最常见的缺乏激素是促肾上腺皮质激素(ACTH)(67%),其次是促甲状腺激素(53%)和促性腺激素(40%)。影像学显示 87%的患者有明确的鞍内增强肿块,33%的患者有垂体柄增粗。3 例行手术治疗。在连续监测中,其他 10 例有明确鞍内肿块的患者,未经免疫抑制治疗,鞍内肿块自行消退或消失。
我们报告的病例中,女性与男性的比例更高,围产期病例的发生率更低。占位效应的症状是最常见的表现,而 ACTH 是最常见缺乏的激素。很少需要手术,大多数患者的肿块自发消退。