Primary lymphocytic hypophysitis: Clinical characteristics and treatment of 50 cases in a single centre in China over 18years
Primary lymphocytic hypophysitis: Clinical characteristics and treatment of 50 cases in a single centre in China over 18years
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DOI：
10.1111/cen.13354
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发表时间：
2017-08-01
影响因子：
3.2
通讯作者：
Zhu, Huijuan
中科院分区：
文献类型：
--
作者：
Wang, Shuchang;Wang, Linjie;Zhu, Huijuan
原发性淋巴细胞性垂体炎（LYH）是罕见的，它往往是在一个小的病例系列评估。本研究旨在描述一个更大队列中原发性LYH的诊断和治疗。设计一项回顾性研究，对1999年至2016年在北京协和医院进行的原发性LYH的诊断和治疗进行了回顾性研究。28例经组织学诊断，22例经临床诊断，符合入选标准。治疗和结果进行了评估。有序Logistic回归分析被用来评估临床参数和结果之间的关联（例如，垂体功能的改善，在MRI上的病灶大小和疾病复发的回归）。低促性腺激素性性腺功能减退是垂体前叶功能障碍最常见的表现（60.0%）;肾上腺功能不全是第三常见的表现（26.0%）; IGF-1轴缺陷最不常见（22.0%）。垂体柄增厚是最常见的影像学表现（96.0%），78.0%的患者表现为鞍内和鞍上扩张。药理剂量的糖皮质激素与垂体前叶功能改善的几率显著相关。结论中国原发性LYH患者垂体前叶功能缺陷的顺序不典型（LH/FSH>TSH>ACTH>IGF-1轴缺陷）。药理剂量的糖皮质激素与改善垂体前叶功能不全显著相关。
ObjectivePrimary lymphocytic hypophysitis (LYH) is rare, and it is often evaluated in a small case series. This study aimed to describe the diagnosis and treatment of primary LYH in a larger cohort.DesignA retrospective study of the diagnosis and treatment of primary LYH was conducted at Peking Union Medical College Hospital from 1999 to 2016.PatientsFifty patients (28 histologically diagnosed and 22 clinically-diagnosed) were eligible for inclusion.MeasurementsClinical, endocrine, pathological and imaging findings; therapies and outcomes were assessed. Ordinal logistic regression analysis was used to evaluate the association between the clinical parameters and outcomes (eg, improvements in pituitary function, regression of lesion size on MRI and disease recurrence).ResultsCentral diabetes insipidus (CDI) (72.0%) was the most common endocrine dysfunction. Hypogonadotropic hypogonadism was the most frequently observed (60.0%) manifestation of anterior pituitary dysfunction; adrenal insufficiency was the third most common (26.0%) manifestation; and IGF-1 axis defects were the least frequent (22.0%). Thickening of the pituitary stalk was the most frequent (96.0%) imaging finding, and 78.0% of the patients exhibited both intrasellar and suprasellar expansion. Pharmacological dose of glucocorticoids was identified to be significantly associated with increased odds of anterior pituitary function improvement. No observed covariates were significantly associated with improvement of CDI and recurrence.ConclusionThe sequence of anterior pituitary deficiencies in Chinese primary LYH patients was atypical (LH/FSH>TSH>ACTH>IGF-1 axis deficiency). A pharmacological dose of glucocorticoids was significantly associated with the improved anterior pituitary insufficiency.