[Clinical aspects of pregnancy-related lymphocytic hypophysitis]

Zhonghua Yi Xue Za Zhi. 2020 Nov 17;100(42):3314-3318. doi: 10.3760/cma.j.cn112137-20200331-01022.
[Article in Chinese]

Abstract

Objective: To analyze clinical features, diagnosis, treatment and prognosis of pregnancy-related lymphocytic hypophysitis (LyH). Methods: The clinical data of 16 cases diagnosed as pregnancy-related LyH at Chinese PLA General Hospital between October 2010 and November 2019 were reviewed. Results: Sixteen patients were included (aged 20-40 years). All patients' symptoms occurred from the last 2 months of pregnancy to 12 months postpartum, with 6 cases in the third trimester and 10 cases during postpartum, and all the patients were diagnosed after delivery. Six patients had lymphocytic adenohypophysitis (LAH), 4 patients had lymphocytic infundibuloneurohypophysitis (LINH), 4 patients had lymphocytic panhypophysitis (LPH), and 2 had lymphocytic hypothalamitis. Eight patients presented with symptoms of intracranial space-occupying lesions, 14 patients had symptoms of anteriorpituitary hormone deficiencies, 9 patients had central diabetes insipidus (CDI), and 2 had hyperprolactinemia. Pituitary MRI showed that the pituitary presented with diffuse enlargement, pituitary stalk thickening, disappearing of high-intensity signals in posterior pituitary and space-occupying lesions in the infundibulum of hypothalamus. Nine patients were treated with immunosuppressive agent, 3 patients alleviated the space-occupying effects after surgery, and 4 patients recovered spontaneously. Fourteen patients were followed up with a period of 3-98 months. Four patients had a relapse, 2 patients had a complete remission, and 12 patients needed long-term hormone replacement therapy. Conclusions: Clinical manifestations of pregnancy-related LyH are diverse. LyH should be suspected in pregnant or postpartum women with a sellar mass to avoid missed diagnosis or misdiagnosis. Immunosuppressant therapy is effective. Overall, LyH patients have a favorable prognosis.

目的: 分析妊娠相关淋巴细胞性垂体炎(LyH)的临床特点、诊治及预后,提高对该病的认识。 方法: 回顾性分析2010年10月至2019年11月解放军总医院第一医学中心收治的妊娠相关LyH患者临床资料。 结果: 共纳入16例患者,年龄20~40岁。发病时间为产前2个月至产后12个月,产前6例,产后10例,就诊时间均为产后。其中淋巴细胞性腺垂体炎6例,淋巴细胞性漏斗-神经垂体炎(LINH)4例,淋巴细胞性全垂体炎4例,淋巴细胞性下丘脑炎2例。16例患者中,8例有肿块占位效应,14例有垂体前叶功能减退症状,9例表现为中枢性尿崩症,2例产后高催乳素血症。垂体磁共振成像可表现垂体弥漫增大、垂体柄增粗、垂体后叶高信号消失、下丘脑漏斗区占位病变。免疫抑制剂治疗9例,3例行手术缓解占位效应,自发缓解4例。共14例获得随访,随访时间3~98个月,其中4例复发,完全缓解2例,12例长期激素替代治疗。 结论: 妊娠相关LyH临床表现呈异质性,孕期及产后的鞍区占位性病变需考虑到LyH的可能,避免误诊和漏诊,免疫抑制剂治疗有效,总体预后良好。.

Keywords: Diagnosis; Disease attributes; Lymphocytic hypophysitis; Pregnancy; Prognosis.

MeSH terms

  • Adult
  • Autoimmune Hypophysitis* / diagnosis
  • Female
  • Humans
  • Hypopituitarism*
  • Magnetic Resonance Imaging
  • Pituitary Diseases* / diagnosis
  • Pituitary Gland
  • Pregnancy
  • Young Adult