Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China.
Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China.
World Neurosurg. 2019 Jul;127:e22-e29. doi: 10.1016/j.wneu.2019.01.250. Epub 2019 Feb 18.
Lymphocytic hypophysitis (LYH) is a rare autoimmune inflammatory disease of the pituitary gland. In this study, we aim to characterize LYH at presentation and focus on the management and prognosis of LYH.
A retrospective study of patients with LYH was conducted between 2011 and 2018 at a single institute. The patients were included by pathologic conformation and strict exclusion criteria. Clinical profile, imaging, and management data were collected.
Twenty patients with LYH (16 women and 4 men) were included. Ten patients were diagnosed histologically and the remaining 10 patients were confirmed clinically of exclusion criteria. The median age at diagnosis was 37 years (range, 16-58 years). Presenting symptoms were followed by polyuria/polydipsia (11, 55%), vision changes (10, 50%), headache (8, 40%), menstrual irregularities and amenorrhea (4, 20%), diplopia (1, 5%), or sexual dysfunction (1, 5%). Eight patients had partial anterior pituitary hormone dysfunction. The thyroid-stimulating hormone axis was most involved. Ten patients received transsphenoidal surgery, 5 patients experienced steroid pulse therapy, and observation was performed on 5 patients. Only 5 patients (25%) showed improvement of anterior pituitary dysfunction after initial management. Recovery of diabetes insipidus occurred in 2 patients (18%). The overall recurrence rate was 22.2%.
Nonoperative treatment is a better option for most patients with LYH because it is effective and noninvasive. Surgery is recommended for definitive diagnosis, severe or rapid progression of neurologic impairment, and glucocorticoid insensitivity. Periodic follow-up is mandatory in a patient's long-term management.
淋巴细胞性垂体炎（LYH）是一种罕见的垂体自身免疫性炎症性疾病。本研究旨在描述 LYH 的临床表现，并重点关注 LYH 的治疗和预后。
对 2011 年至 2018 年期间在一家医院就诊的 LYH 患者进行了回顾性研究。通过病理证实并严格排除标准纳入患者。收集了临床特征、影像学和治疗数据。
共纳入 20 例 LYH 患者（16 例女性和 4 例男性）。10 例患者经组织学诊断，其余 10 例患者根据排除标准临床确诊。诊断时的中位年龄为 37 岁（范围 16-58 岁）。主要表现为多尿/多饮（11 例，55%）、视力改变（10 例，50%）、头痛（8 例，40%）、月经不规律和闭经（4 例，20%）、复视（1 例，5%）或性功能障碍（1 例，5%）。8 例患者存在部分垂体前叶激素功能障碍，其中甲状腺刺激激素轴最易受累。10 例患者接受了经蝶窦手术，5 例患者接受了类固醇冲击治疗，5 例患者仅接受了观察。仅有 5 例（25%）患者在初始治疗后垂体前叶功能障碍得到改善。2 例（18%）患者恢复了尿崩症。总的复发率为 22.2%。
对于大多数 LYH 患者，非手术治疗是更好的选择，因为它有效且无创。手术适用于明确诊断、神经功能损害严重或进展迅速以及对糖皮质激素不敏感的患者。在患者的长期管理中，必须进行定期随访。
