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淋巴细胞性垂体炎

Lymphocytic hypophysitis.

作者信息

Molitch Mark E, Gillam Mary P

机构信息

Division of Endocrinology, Metabolism, and Molecular Medicine, Feinberg School of Medicine, Northwestern University, Chicago, Ill. 60611, USA.

出版信息

Horm Res. 2007;68 Suppl 5:145-50. doi: 10.1159/000110611. Epub 2007 Dec 10.

DOI:10.1159/000110611
PMID:18174733
Abstract

BACKGROUND

Lymphocytic hypophysitis is a disorder of the pituitary gland that presents as a sellar mass lesion and/or hypopituitarism. It causes pituicyte destruction and hypopituitarism and is speculated to have an autoimmune basis.

DIAGNOSIS

Lymphocytic hypophysitis should be considered in the differential diagnosis of pituitary masses and/or hypopituitarism in females who are pregnant or in the early postpartum period, especially in cases associated with other autoimmune diseases or unusual patterns of hormone deficiencies. A definitive diagnosis requires tissue biopsy. A presumptive clinical diagnosis can be made based on a history of gestational or postpartum hypopituitarism, a contrast-enhancing sellar mass with imaging features characteristic of lymphocytic hypophysitis, a pattern of pituitary hormone deficiency with early loss of adrenocorticotrophic hormone and thyroid-stimulating hormone unlike that typically found with macroadenomas, relatively rapid development of hypopituitarism and a degree of pituitary failure disproportionate to the size of the mass. Symptoms resulting from partial or panhypopituitarism occur in approximately 80% of cases and multiple deficiencies are found in approximately 75% of cases.

MANAGEMENT

Appropriate management remains controversial. Corticosteroid therapy has been advocated as a means of attenuating inflammation, but given the uncertainty of its efficacy and the known adverse effects, such therapy does not seem justified for most patients. The optimal surgical strategy involves partial resection of the mass to decompress the surrounding structures. All patients with lymphocytic hypophysitis require appropriate replacement therapy for deficient hormones. Long-term follow-up is mandatory to monitor for the development of other hormonal deficits.

摘要

背景

淋巴细胞性垂体炎是一种垂体疾病,表现为鞍区占位性病变和/或垂体功能减退。它导致垂体细胞破坏和垂体功能减退,据推测有自身免疫基础。

诊断

对于妊娠或产后早期的女性出现垂体肿块和/或垂体功能减退的情况,尤其是伴有其他自身免疫性疾病或激素缺乏异常模式的病例,应考虑淋巴细胞性垂体炎。明确诊断需要组织活检。根据妊娠或产后垂体功能减退病史、具有淋巴细胞性垂体炎特征性影像学表现的增强鞍区肿块、与大腺瘤不同的促肾上腺皮质激素和促甲状腺激素早期缺失的垂体激素缺乏模式、垂体功能减退相对快速的发展以及垂体功能衰竭程度与肿块大小不成比例等情况,可做出临床推测诊断。约80%的病例出现部分或全垂体功能减退症状,约75%的病例存在多种激素缺乏。

治疗

合适的治疗方法仍存在争议。有人主张使用皮质类固醇疗法来减轻炎症,但鉴于其疗效的不确定性和已知的不良反应,这种疗法对大多数患者似乎并不合理。最佳手术策略是部分切除肿块以减压周围结构。所有淋巴细胞性垂体炎患者都需要对缺乏的激素进行适当的替代治疗。必须进行长期随访以监测其他激素缺乏的发展情况。

相似文献

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Lymphocytic hypophysitis.淋巴细胞性垂体炎
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How reliably can autoimmune hypophysitis be diagnosed without pituitary biopsy.不进行垂体活检,自身免疫性垂体炎的诊断可靠性如何。
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