Objective: To analyze clinical features, prognosis and treatment of lymphocytic hypophysitis (LYH). Methods: The clinical data, treatments and outcomes of 18 cases diagnosed as LYH at Chinese PLA General Hospital between January 2001 and July 2017 was respectively reviewed. Results: Eighteen patients with histology-proven LYH (13 females and 5 males ) were identified. All lymphocytic adenohypophysitis (LAH) were females(n=6), two of whom were associated with pregnancy. Eleven patients (6 females and 5 males) had lymphocytic panhypophysitis (LPH) and one(female) had hypothalamitis. Pre-treatment evaluation revealed that 11 patients presented with symptoms of intracranial space-occupying lesions, 12 patients had symptoms of anterior pituitary hormone deficiencies, and 12 patients had central diabetes insipidus (CDI). All patients had space-occupying lesions on magnetic resonance imaging (MRI), which were symmetrically enlarged and homogenously enhanced with or without pituitary stalk thickening. Before or after surgery, 11 patients received immunosuppressant therapy or radiotherapy to alleviate space-occupying effect. After 4-204 months follow-up, 5 patients had a relapse and received immunosuppressants, radiotherapy or surgery to achieve remission. Full recovery (both symptomatic and radiographic) was seen in 6 patients, and 11 patients maintained stable replacement therapy. Conclusions: LYH presents with acute space-occupying effects such as headache, visual disturbances, hypopituitarism, CDI and mild hyperprolactinemia, especially with characteristic radiographic manifestations. Usually, surgery reliably establishes diagnosis, and immunosuppressant therapy is a necessity. On the whole, LYH has a good prognosis.
目的： 总结原发性淋巴细胞性垂体炎(LyH)的临床特点、治疗和预后，提高诊治水平。 方法： 回顾性分析2001年1月至2017年7月于解放军总医院病理确诊的18例LyH的临床数据、诊疗转归。 结果： 本研究共入选18例病理诊断为LyH的患者(男5例，女13例)，其中淋巴细胞性腺垂体炎(LAH)6例，均为女性，2例在妊娠期起病；淋巴细胞性全垂体炎(LPH)11例，男5例，女6例；淋巴细胞性下丘脑炎1例。11例患者有占位性症状，12例有中枢性尿崩症(CDI)，12例有垂体前叶功能减退症。磁共振成像(MRI)表现为垂体对称性增大伴均匀强化，伴或不伴垂体柄增粗。11例患者术前或术后接受免疫抑制剂或放疗以减轻占位效应。18例患者手术后随访时间4～204个月，其中有5例复发，给予免疫抑制剂、放疗或再次手术后缓解。6例患者完全缓解，11例患者长期给予靶腺激素替代治疗。 结论： LyH多有急性占位症状如头痛、视力视野障碍、垂体前叶功能减退伴CDI和轻度高催乳素血症，伴典型的MRI表现；手术主要用于确诊疾病，多需辅以免疫抑制剂治疗；LyH总体预后良好。.
Keywords: Anterior pituitary dysfunction; Central diabetes insipidus; Lymphocytic hypophysitis; Pathology; Prognosis.