淋巴细胞性垂体炎三例并文献复习

目的提高临床医师对淋巴细胞性垂体炎的认识。方法分析3例淋巴细胞性垂体炎的临床特点和病理结果。结果3例患者均为青年女性,例1产后起病,例2和例3在非妊娠或产后期间发病;影像学特点:病变广泛,不仅局限于鞍区和垂体柄,并向下丘脑扩展呈“舌状”改变侵犯海绵窦;增强后病变均匀或环状强化;主要临床表现:(1)垂体功能受损和尿崩症;(2)有局部受压症状,如头痛,视交叉受压;(3)病变侵犯海绵窦,引起动眼、外展神经麻痹;2例经病理证实为淋巴细胞性垂体炎,垂体组织见大量淋巴细胞、浆细胞、组织细胞浸润;2例大剂量甲泼尼龙治疗后,症状明显好转。结论淋巴细胞性垂体炎亦可发生在非妊娠或产后期间的年轻女性,大剂量甲泼尼龙冲击治疗能有效缩小病变和改善垂体功能。

Lymphocytic hypophysitis: a report of 3 cases

Objective Lymphocytic hypophysitis is a rare inflammatory lesion of pituitary gland. To enhance the knowledge of lymphocytic hypophysitis,herein we reported 3 cases of lymphocytic hypophysitis and reviewed the literature. Methods The clinical data of 3 patients diagnosed as having lymphocytic hypophysitis were analysed.Results All the three patients were young females, one of them(case 1)was affected in the postpartum period,however,the others were neither pregnant nor in postpartum period. Clinically, the most frequently seen symptoms and signs were attributable to pituitary hypofunction, headache and diabetes insipidus. Pituitary MRI revealed enhanced mass with pituitary stalk enlargement expanding the pituitary fossa, extending into suprasellar area and compressing optic chiasm. Typically the lesion appeared hypointense or isointense on T1-weighted imaging, but hyperintense on T2-weighted imaging. Histopathological examination showed extensive destruction of anterior acini of the pituitary with a dense infiltration of lymphocytes, plasma cells, histocytes and other inflammatory cells. Two patients(case 2 and 3)were successfully treated for mass reduction of pituitary gland and restoration of pituitary function with high dose methylprednisolone pulse therapy(HDMPT).Conclusions Lymphocytic hypophysitis is a rare autoimmune endocrinopathy which can affect young woman in the postpartum period, or in the peripartum period, characterized by focal or extensive lymphocytic infiltration of anterior pituitary acini. It may cause pituitary expansion and a varying degree of hypopituitarism mimicking the features observed in pituitary adenoma. HDMPT was proved to be effective for mass reduction of pituitary gland and restoration of pituitary function.