Yang Lian Zi, Velaiutham Shanty
Endocrine Unit, Penang General Hospital, Pulau, Pinang, Malaysia.
J ASEAN Fed Endocr Soc. 2025 May;40(1):121-125. doi: 10.15605/jafes.040.01.08. Epub 2025 Apr 20.
Lymphocytic hypophysitis (LHy) is a rare autoimmune inflammatory process that selectively affects the neurohypophysis and infundibulum, typically presenting with arginine vasopressin deficiency (AVP-D). On magnetic resonance imaging (MRI) with contrast, there is a thickening of the pituitary stalk, enlargement of the neurohypophysis or both with homogeneous enhancement. LHy can be self-limiting and regression can be seen radiologically during follow-up. A 22-year-old male presented with clinical findings consistent with AVP-D in 2016. MRI brain demonstrated enlargement of the pituitary stalk and absence of a posterior pituitary bright spot. He was given a trial of glucocorticoid treatment. His serial MRI brain showed a reduction of the pituitary stalk, but the AVP-D persisted. He was diagnosed with LHy. LHy is characterized by lymphocytic infiltration, leading to eventual destruction of the pituitary tissue accompanied by varying degrees of pituitary dysfunction. Definite diagnosis can only be established via pituitary stalk biopsy. Due to the wide range of possible aetiologies, close monitoring is strongly recommended for the treatment of presumed cases lacking histopathologic confirmation. The response rate to glucocorticoids has been variable. Periodic monitoring of anterior pituitary function and pituitary MRI are essential in the management of this condition.
淋巴细胞性垂体炎（LHy）是一种罕见的自身免疫性炎症过程，选择性地影响神经垂体和漏斗部，典型表现为精氨酸血管加压素缺乏（AVP-D）。在增强磁共振成像（MRI）上，垂体柄增厚，神经垂体增大或两者均有，且呈均匀强化。LHy可自限，随访期间影像学上可见病变消退。一名22岁男性于2016年出现与AVP-D一致的临床表现。脑部MRI显示垂体柄增粗，垂体后叶亮点消失。给予糖皮质激素治疗试验。他的系列脑部MRI显示垂体柄变细，但AVP-D持续存在。他被诊断为LHy。LHy的特征是淋巴细胞浸润，导致垂体组织最终破坏，并伴有不同程度的垂体功能障碍。明确诊断只能通过垂体柄活检来确立。由于可能的病因范围广泛，对于缺乏组织病理学证实的疑似病例，强烈建议密切监测其治疗情况。对糖皮质激素的反应率各不相同。定期监测垂体前叶功能和垂体MRI对这种疾病的管理至关重要。
