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垂体炎的临床表现与管理:病例系列观察研究

Clinical presentation and management of hypophysitis: An observational study of case series.

作者信息

Karrou Marouan, Benyakhlef Salma, Alla Achwak, Messaoudi Najoua, Amar Asmae Oulad, Rouf Siham, Kamaoui Imane, Oulali Noureddine, Moufid Faycal, Abda Naima, Latrech Hanane

机构信息

Department of Endocrinology-Diabetology and Nutrition, Medical School, Mohammed VI University Hospital Centre, Oujda, Oujda-Angad, Morocco.

Department of Radiology, Medical School, Mohammed VI University Hospital Centre, Oujda, Oujda-Angad, Morocco.

出版信息

Surg Neurol Int. 2021 Jun 28;12:304. doi: 10.25259/SNI_454_2021. eCollection 2021.

DOI:10.25259/SNI_454_2021
PMID:34345445
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8326108/
Abstract

BACKGROUND

Hypophysitis is described as a rare chronic inflammatory affection of the pituitary gland. However, to date, its pathogenesis has not been completely cleared up. Clinical features are polymorphic, including symptoms related to inflammatory compression and/or hypopituitarism. Laboratory tests determine hormone deficiencies orientating replacement therapy's protocol. MRI of the hypothalamic-pituitary region is crucial in exhibiting major radiological signs such as pituitary homogeneous enlargement and gland stalk's thickening. The etiological diagnosis is still challenging without affecting the management strategy. Corticosteroids have widely been used but a close follow-up without any treatment has also been approved.

CASE DESCRIPTION

In this report, seven patients with hypophysitis have been collected over a period of 6 years. The average age of our patients was 32.1 years ± 11.8 with a female predominance (71.4%). Panhypopituitarism was objective in 42.9% of cases, a combined deficiency of the hypothalamic-pituitary thyroid, adrenal and gonadal axes in 28.6% of cases. A central diabetes insipidus was noted in 42.9% of the patients. Idiopathic hypophysitis was the most common etiology. The use of long course corticosteroids was required in 28.6% when compressive signs were reported.

CONCLUSION

Hypophysitis remains a rare disease with nonspecific clinical and radiological patterns. Autoimmune origin seems to be the most frequent etiology. No guidelines have been established for hypophysitis management and the evolution is still unpredictable.

摘要

背景

垂体炎被描述为一种罕见的垂体慢性炎症性疾病。然而,迄今为止,其发病机制尚未完全明确。临床特征具有多态性,包括与炎症压迫和/或垂体功能减退相关的症状。实验室检查可确定激素缺乏情况,从而指导替代治疗方案。下丘脑 - 垂体区域的磁共振成像(MRI)对于显示主要的放射学征象至关重要,如垂体均匀增大和腺柄增粗。在不影响治疗策略的情况下,病因诊断仍然具有挑战性。皮质类固醇已被广泛使用,但未经任何治疗的密切随访也已得到认可。

病例描述

在本报告中,6年间共收集了7例垂体炎患者。我们患者的平均年龄为32.1岁±11.8岁,女性占优势(71.4%)。42.9%的病例存在全垂体功能减退,28.6%的病例存在下丘脑 - 垂体 - 甲状腺、肾上腺和性腺轴联合缺乏。42.9%的患者出现中枢性尿崩症。特发性垂体炎是最常见的病因。当出现压迫症状时,28.6%的患者需要长期使用皮质类固醇。

结论

垂体炎仍然是一种罕见疾病,具有非特异性的临床和放射学表现。自身免疫起源似乎是最常见的病因。目前尚未建立垂体炎管理指南,其病情发展仍然不可预测。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8f79/8326108/58e450a35fba/SNI-12-304-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8f79/8326108/cf73fb8c5595/SNI-12-304-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8f79/8326108/c863bdbeaa4b/SNI-12-304-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8f79/8326108/58e450a35fba/SNI-12-304-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8f79/8326108/cf73fb8c5595/SNI-12-304-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8f79/8326108/c863bdbeaa4b/SNI-12-304-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8f79/8326108/58e450a35fba/SNI-12-304-g003.jpg

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Hypophysitis: Evaluation and Management.垂体炎:评估与管理
Clin Diabetes Endocrinol. 2016 Sep 6;2:15. doi: 10.1186/s40842-016-0034-8. eCollection 2016.
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The management of hypophysitis.垂体炎的管理
Minerva Endocrinol. 2016 Sep;41(3):390-9. Epub 2016 Mar 10.
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Diagnosis and management of tumor-like hypophysitis: A retrospective case series.肿瘤样垂体炎的诊断与管理:一项回顾性病例系列研究。
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Treatment of Primary Hypophysitis in Germany.德国原发性垂体炎的治疗
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Hypophysitis.垂体炎
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Clinical characteristics, management, and outcome of 22 cases of primary hypophysitis.22 例原发性垂体炎的临床特征、治疗及转归
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The prevalence of IgG4-related hypophysitis in 170 consecutive patients with hypopituitarism and/or central diabetes insipidus and review of the literature.170 例垂体功能减退症和/或中枢性尿崩症患者 IgG4 相关垂体炎的患病率及文献复习。
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