翟笑, 朱惠娟. 自身免疫性垂体炎的诊治进展[J]. 罕见病研究, 2022, 1(1): 45-50. DOI: 10.12376/j.issn.2097-0501.2022.01.008
引用本文: 翟笑, 朱惠娟. 自身免疫性垂体炎的诊治进展[J]. 罕见病研究, 2022, 1(1): 45-50. DOI: 10.12376/j.issn.2097-0501.2022.01.008
ZHAI Xiao, ZHU Huijuan. Progress in Diagnosis and Treatment of Autoimmune Hypophysitis[J]. Journal of Rare Diseases, 2022, 1(1): 45-50. DOI: 10.12376/j.issn.2097-0501.2022.01.008
Citation: ZHAI Xiao, ZHU Huijuan. Progress in Diagnosis and Treatment of Autoimmune Hypophysitis[J]. Journal of Rare Diseases, 2022, 1(1): 45-50. DOI: 10.12376/j.issn.2097-0501.2022.01.008

自身免疫性垂体炎的诊治进展

Progress in Diagnosis and Treatment of Autoimmune Hypophysitis

  • 摘要: 自身免疫性垂体炎是自身免疫介导侵犯垂体的罕见疾病,其中育龄期女性好发的淋巴细胞性垂体炎最常见。常见临床表现包括头痛、视功能障碍、垂体功能减低等,垂体核磁成像可见弥漫增大、可被增强的鞍内占位。病理是自身免疫性垂体炎诊断的金标准,但因手术创伤和可能的垂体功能损害需谨慎选择。多结合患者典型的临床表现、影像学特征进行临床诊断,糖皮质激素治疗有效是自身免疫性垂体炎临床诊断依据之一。足疗程糖皮质激素治疗淋巴细胞垂体炎有良好的治疗效果,有效改善临床症状、缓解占位效应,促进垂体功能恢复。部分类型的自身免疫性垂体炎需手术治疗。自身免疫性垂体炎的发病机制、诊断标记物和治疗靶点有待深入研究。

     

    Abstract: Autoimmune hypophysitis is a rare disorder characterized by autoimmune-mediated inflammation of the pituitary, among which lymphocytic hypophysitis is the most common type that occurs mainly in young women of reproductive ages. Its common clinical manifestation includes headache, visual dysfunction, hypopituitarism and etc. Typical magnetic resonance imaging shows diffuse homogenous enlargement of the pituitary gland with gadolinium enhancement and stalk thickening. Pituitary biopsy with histopathological examination is the gold standard for the diagnosis of autoimmune hypophysitis, but it should be performed with extra care in that transsphenoidal surgery is invasive and may cause pituitary insufficiency. Closely relating to clinical manifestation of the patients and the radiological features is advised for diagnosis. Good response to glucocorticoid therapy also help confirm the diagnosis. Full course of pharmacological glucocorticoid treatment can effectively control the clinical symptoms, reduce the mass effects, and promote the resumption of pituitary functions. Surgery is usually necessary for xanthomatous and granulomatous hypophysitis. Pathophysiological mechanism, diagnostic biomarkers, and need studying further.

     

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