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18例原发性淋巴细胞性垂体炎的临床特征及预后

[Clinical features and prognosis of 18 cases of primary lymphocytic hypophysitis].

作者信息

Wei Q, Yang G Q, Li Y J, Zang L, Pei Y, Du J, Gu W J, Ba J M, Lü Z H, Dou J T, Mu Y M, Lu J M

机构信息

Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.

出版信息

Zhonghua Yi Xue Za Zhi. 2018 Jan 9;98(2):102-108. doi: 10.3760/cma.j.issn.0376-2491.2018.02.006.

DOI:10.3760/cma.j.issn.0376-2491.2018.02.006
PMID:29343033
Abstract

To analyze clinical features, prognosis and treatment of lymphocytic hypophysitis (LYH). 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. Eighteen patients with histology-proven LYH (13 females and 5 males ) were identified. All lymphocytic adenohypophysitis (LAH) were females(=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. 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.

摘要

分析淋巴细胞性垂体炎(LYH)的临床特征、预后及治疗方法。回顾了2001年1月至2017年7月在中国人民解放军总医院确诊为LYH的18例患者的临床资料、治疗方法及预后情况。确诊为组织学证实的LYH患者18例(女性13例,男性5例)。其中淋巴细胞性腺垂体炎(LAH)均为女性(n = 6),2例与妊娠相关。11例患者(女性6例,男性5例)为淋巴细胞性全垂体炎(LPH),1例(女性)为下丘脑炎。治疗前评估显示,11例患者有颅内占位性病变症状,12例患者有垂体前叶激素缺乏症状,12例患者有中枢性尿崩症(CDI)。所有患者磁共振成像(MRI)均显示有占位性病变,病变对称增大,均匀强化,伴或不伴垂体柄增粗。手术前后,11例患者接受免疫抑制治疗或放射治疗以减轻占位效应。经过4 - 204个月的随访,5例患者复发,接受免疫抑制剂、放射治疗或手术治疗后病情缓解。6例患者完全康复(症状和影像学均恢复正常),11例患者维持稳定的替代治疗。LYH表现为急性占位效应,如头痛、视觉障碍、垂体功能减退、CDI和轻度高催乳素血症,尤其是具有特征性的影像学表现。通常,手术可可靠地确诊,免疫抑制治疗是必要的。总体而言,LYH预后良好。

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引用本文的文献

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Clinical presentation and magnetic resonance imaging characteristics of lymphocytic hypophysitis: a systematic review with meta-analysis.淋巴细胞性垂体炎的临床表现及磁共振成像特征:一项荟萃分析的系统评价
Arch Med Sci. 2021 Dec 14;19(4):976-986. doi: 10.5114/aoms/144628. eCollection 2023.