Tao Tingting, Zhang Ziyi, Li Hong
Department of Endocrinology, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China.
Neuro Endocrinol Lett. 2018 Mar;39(1):43-48.
Lymphocytic hypophysitis (LH) is a rare inflammatory disorder involving the pituitary gland, often with other autoimmune diseases combined. The coexistence of LH and Behçet's disease (BD) is a rare combination and only one case was reported in the previous literatures. A 50-year-old man was admitted into Sir Run Run Shaw Hospital presented with frontal headache and fatigue which had lasted for four months. Endocrinological inspection indicated anterior pituitary dysfunction. Magnetic resonance imaging (MRI) revealed homogeneous pituitary enlargement and thickened pituitary stalk. Administration of glucocorticoids could effectively relieve headache and reduced pituitary mass volume. Oral aphthosis, skin lesions and positive pathergy tests were recognized later, which were characteristic features of BD. Although the diagnosis of BD is mainly dependent on clinical manifestations, PT is still a useful diagnostic tool 0f high specificityfor BD. And this male patient was diagnosed with both LH and BD afterwards. Then he was treated with cyclophosphamide and medium doses of methylprednisolone and remained in good conditions at the follow-up. LH and BD might share a common underlying autoimmune pathogenesis. The presentation of endocrinologic disturbances such as anterior pituitary dysfunction with typical features of skin lesions should prompt further investigation of possible comorbid autoimmune disease involving multiple organ systems. Early diagnosis and close monitoring are vitally important to ensure a stable endocrinologic status.
淋巴细胞性垂体炎(LH)是一种罕见的累及垂体的炎症性疾病,常合并其他自身免疫性疾病。LH与白塞病(BD)并存是一种罕见的组合,既往文献仅报道过1例。一名50岁男性因持续4个月的前额头痛和疲劳入住邵逸夫医院。内分泌检查显示垂体前叶功能障碍。磁共振成像(MRI)显示垂体均匀增大,垂体柄增粗。给予糖皮质激素可有效缓解头痛并缩小垂体肿块体积。随后出现口腔溃疡、皮肤病变和针刺反应阳性,这些都是BD的特征性表现。虽然BD的诊断主要依赖于临床表现,但针刺试验仍是诊断BD的一种具有高特异性的有用工具。该男性患者随后被诊断为LH和BD。然后他接受了环磷酰胺和中等剂量甲泼尼龙治疗,随访时情况良好。LH和BD可能有共同的自身免疫发病机制。出现内分泌紊乱,如垂体前叶功能障碍并伴有典型的皮肤病变特征,应促使进一步调查可能合并的累及多器官系统的自身免疫性疾病。早期诊断和密切监测对于确保稳定的内分泌状态至关重要。