Abstract

Hypophysitis is defined as inflammation of the pituitary gland that is primary or secondary to a local or systemic process. Differential diagnosis is broad (including primary tumors, metastases, and lympho-proliferative diseases) and multifaceted. Patients with hypophysitis typically present with headaches, some degree of anterior and/or posterior pituitary dysfunction, and enlargement of pituitary gland and/or stalk, as determined by imaging. Most hypophysitis causes are autoimmune, but other etiologies include inflammation secondary to sellar tumors or cysts, systemic diseases, and infection or drug-induced causes. Novel pathologies such as immunoglobulin G4-related hypophysitis, immunotherapy-induced hypophysitis, and paraneoplastic pituitary-directed autoimmunity are also included in a growing spectrum of this rare pituitary disease. Typical magnetic resonance imaging reveals stalk thickening and homogenous enlargement of the pituitary gland; however, imaging is not always specific. Diagnosis can be challenging, and ultimately, only a pituitary biopsy can confirm hypophysitis type and rule out other etiologies. A presumptive diagnosis can be made often without biopsy. Detailed history and clinical examination are essential, notably for signs of underlying etiology with systemic manifestations. Hormone replacement and, in selected cases, careful observation is advised with imaging follow-up. High-dose glucocorticoids are initiated mainly to help reduce mass effect. A response may be observed in all auto-immune etiologies, as well as in lymphoproliferative diseases, and, as such, should not be used for differential diagnosis. Surgery may be necessary in some cases to relieve mass effect and allow a definite diagnosis. Immunosuppressive therapy and radiation are sometimes also necessary in resistant cases.

中文翻译：

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垂体炎，一种越来越多的罕见垂体疾病谱

摘要

垂体炎定义为原发于或继发于局部或全身过程的垂体炎症。鉴别诊断是广泛的（包括原发性肿瘤、转移和淋巴增殖性疾病）和多方面的。垂体炎患者通常表现为头痛、一定程度的垂体前叶和/或后叶功能障碍，以及垂体和/或垂体垂体扩大，如影像学检查所示。大多数垂体炎原因是自身免疫性，但其他病因包括继发于鞍区肿瘤或囊肿的炎症、全身性疾病以及感染或药物诱导的原因。免疫球蛋白 G4 相关性垂体炎、免疫治疗诱导的垂体炎和副肿瘤性垂体定向自身免疫等新病理也包括在这种罕见垂体疾病的范围内。典型的磁共振成像显示垂体的茎增厚和均匀增大；然而，成像并不总是具体的。诊断可能具有挑战性，最终，只有垂体活检才能确定垂体炎类型并排除其他病因。通常无需活检即可做出推测性诊断。详细的病史和临​​床检查是必不可少的，特别是对于具有全身表现的潜在病因的迹象。激素替代治疗，在某些情况下，建议在影像学随访中仔细观察。开始使用大剂量糖皮质激素主要是为了帮助减少占位效应。在所有自身免疫病因以及淋巴组织增生性疾病中都可以观察到反应，因此不应用于鉴别诊断。在某些情况下可能需要进行手术以缓解占位效应并做出明确诊断。在耐药病例中有时也需要免疫抑制治疗和放射治疗。