Iuliano Sherry L, Laws Edward R
a Brigham and Women's Hospital, Pituitary/Neuroendocrine Center, 75 Francis Street, Boston, MA 02115, USA.
b
Expert Rev Endocrinol Metab. 2011 Nov;6(6):777-783. doi: 10.1586/eem.11.74.
Although a relatively rare phenomenon, lymphocytic hypophysitis can represent a difficult diagnostic and therapeutic challenge. This condition was initially thought to be an autoimmune phenomenon occurring primarily in women in the postpartum state. It is now recognized as a disorder that can affect both men and women over a rather large age range, from young adults to the elderly, and may be linked to a number of autoimmune phenomena. We have reviewed the literature on lymphocytic hypophysitis, and added the detailed analysis of seven cases of presumed lymphocytic hypophysitis from our own experience. The case studies demonstrate the wide spectrum of manifestations of this disorder, the differential diagnosis and the rationale and the nuances of treatment. Diagnostic studies, including laboratory evaluation and the characteristic MRIs, are reviewed. The histopathology and results of special stains are also presented, emphasizing the characteristic pathologic findings in this spectrum of pituitary disease. Our experience and that of others demonstrates that this condition has a characteristic presentation - that is, headache, hypopituitarism and diabetes insipidus. When significant mass effect is present, suprasellar and parasellar extension may produce visual loss and/or diplopia. Treatment strategies and outcomes are given for each of the seven patients. Corticosteroids are recommended as first-line management, and are usually successful. Relapses requiring additional therapy, however, are not uncommon. In such cases, trans-sphenoidal surgery, other more aggressive forms of medical therapy and rarely, radiosurgery can also be employed. Lymphocytic hypophysitis is a challenging entity. With accurate diagnosis and sequential, expertly managed therapeutic measures, it can be controlled. Continued surveillance of the patients is essential, as relapses may occur.
尽管淋巴细胞性垂体炎是一种相对罕见的现象，但它可能带来诊断和治疗方面的难题。这种病症最初被认为是一种主要发生在产后女性的自身免疫现象。现在人们认识到它是一种可影响从年轻人到老年人这一相当大年龄范围的男性和女性的疾病，并且可能与多种自身免疫现象有关。我们回顾了关于淋巴细胞性垂体炎的文献，并结合我们自己的经验对7例疑似淋巴细胞性垂体炎病例进行了详细分析。这些病例研究展示了这种疾病广泛的临床表现、鉴别诊断以及治疗的基本原理和细微差别。文中回顾了包括实验室评估和特征性磁共振成像在内的诊断研究。还展示了组织病理学和特殊染色结果，强调了这一系列垂体疾病的特征性病理表现。我们和其他人的经验表明，这种病症有典型的表现，即头痛、垂体功能减退和尿崩症。当出现明显的占位效应时，鞍上和鞍旁扩展可能导致视力丧失和/或复视。文中给出了7例患者各自的治疗策略和结果。推荐使用皮质类固醇作为一线治疗方法，通常会取得成功。然而，需要额外治疗的复发情况并不少见。在这种情况下，也可采用经蝶窦手术、其他更积极的药物治疗方式，很少情况下还可采用放射外科治疗。淋巴细胞性垂体炎是一个具有挑战性的疾病。通过准确诊断和有序、专业管理的治疗措施，病情可以得到控制。对患者进行持续监测至关重要，因为可能会复发。
