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一名原发性垂体炎患者在接受糖皮质激素治疗后完全缓解。

Complete remission after glucocorticoid therapy in a patient with primary hypophysitis.

作者信息

Hacioglu Aysa, Ekinci Gazanfer, Karaca Zuleyha, Türe Uğur, Kelestimur Fahrettin

机构信息

Department of Endocrinology, Erciyes University Medical School, Kayseri, Turkey.

Department of Radiology, Yeditepe University Medical School, Istanbul, Turkey.

出版信息

Endocrinol Diabetes Metab Case Rep. 2024 Oct 7;2024(4). doi: 10.1530/EDM-23-0125. Print 2024 Oct 1.

DOI:10.1530/EDM-23-0125
PMID:39393406
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11466270/
Abstract

SUMMARY

Primary hypophysitis is a rare disease that may have variable clinical presentations. The main treatment options are clinical observation, immunosuppressive drugs, and surgery. Glucocorticoids are used as first-line medical therapy; however, non-responsiveness and recurrences are the major problems. We present a 30-year-old male patient who had an excellent radiologic response to a single course of glucocorticoids. The patient presented with malaise and severe headaches of acute onset. Cranial MRI revealed a pituitary mass compressing the optic chiasm. Hormonal evaluation studies were consistent with anterior pituitary hormone dysfunction except for the growth hormone axis. There was a mild compression on the optic chiasm in the pituitary MRI. The patient was started on methylprednisolone therapy at a dose of 80 mg/day. The pituitary MRI revealed complete regression of the mass after 2 months, and there was a complete recovery of pituitary functions after 6 months. There is no consensus on the optimal dose and duration of glucocorticoid therapy for primary hypophysitis in the literature. We report that steroid therapy, even in lower doses, might be effective in mild-to-moderate cases.

LEARNING POINTS

Primary hypophysitis is a rare disease with a varied clinical course, and hence the treatment strategies should be individualized. There is no consensus on the optimal dose and duration of glucocorticoid therapy. Glucocorticoid therapy may induce complete remission in some patients, especially with a mild-to-moderate disease course and during the acute phase of the disease.

摘要

摘要

原发性垂体炎是一种罕见疾病,临床表现可能多种多样。主要治疗选择包括临床观察、免疫抑制药物和手术。糖皮质激素用作一线药物治疗;然而,无反应和复发是主要问题。我们报告一名30岁男性患者,其对单疗程糖皮质激素有良好的影像学反应。该患者急性起病,表现为不适和严重头痛。头颅磁共振成像(MRI)显示垂体肿块压迫视交叉。激素评估研究显示,除生长激素轴外,与垂体前叶激素功能障碍一致。垂体MRI显示视交叉有轻度受压。患者开始接受甲泼尼龙治疗,剂量为80毫克/天。2个月后垂体MRI显示肿块完全消退,6个月后垂体功能完全恢复。文献中对于原发性垂体炎糖皮质激素治疗的最佳剂量和疗程尚无共识。我们报告,即使是较低剂量的类固醇治疗,在轻至中度病例中可能也是有效的。

学习要点

原发性垂体炎是一种临床病程多样的罕见疾病,因此治疗策略应个体化。对于糖皮质激素治疗的最佳剂量和疗程尚无共识。糖皮质激素治疗可能使一些患者完全缓解,尤其是在疾病病程为轻至中度以及疾病急性期。

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本文引用的文献

1
Glucocorticoid therapy as first-line treatment in primary hypophysitis: a systematic review and individual patient data meta-analysis.糖皮质激素治疗作为原发性垂体炎的一线治疗:一项系统评价和个体患者数据荟萃分析。
Endocr Connect. 2022 Dec 22;12(2). doi: 10.1530/EC-22-0311. Print 2023 Feb 1.
2
Outcomes of Initial Management Strategies in Patients With Autoimmune Lymphocytic Hypophysitis: A Systematic Review and Meta-analysis.自身免疫性淋巴细胞性垂体炎患者初始治疗策略的结局:系统评价和荟萃分析。
J Clin Endocrinol Metab. 2022 Mar 24;107(4):1170-1190. doi: 10.1210/clinem/dgab839.
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Hypophysitis: A comprehensive overview.
垂体炎:全面概述。
Presse Med. 2021 Dec;50(4):104076. doi: 10.1016/j.lpm.2021.104076. Epub 2021 Oct 21.
4
Hypophysitis, the Growing Spectrum of a Rare Pituitary Disease.垂体炎,一种罕见垂体疾病的不断扩展的范畴。
J Clin Endocrinol Metab. 2022 Jan 1;107(1):10-28. doi: 10.1210/clinem/dgab672.
5
Early Pulse Glucocorticoid Therapy and Improved Hormonal Outcomes in Primary Hypophysitis.原发性垂体炎中早期脉冲糖皮质激素治疗与激素结局改善相关。
Neuroendocrinology. 2022;112(2):186-195. doi: 10.1159/000516006. Epub 2021 Mar 19.
6
Hypophysitis (Including IgG4 and Immunotherapy).垂体炎(包括 IgG4 和免疫疗法)。
Neuroendocrinology. 2020;110(9-10):822-835. doi: 10.1159/000506903. Epub 2020 Mar 4.
7
Hypophysitis: An update on the novel forms, diagnosis and management of disorders of pituitary inflammation.垂体炎:新型垂体炎症性疾病的形式、诊断和治疗方法的最新进展。
Best Pract Res Clin Endocrinol Metab. 2019 Dec;33(6):101371. doi: 10.1016/j.beem.2019.101371. Epub 2019 Dec 12.
8
Primary hypophysitis: Experience of a Single Tertiary Center.原发性垂体炎:一家三级中心的经验
Exp Clin Endocrinol Diabetes. 2021 Jan;129(1):14-21. doi: 10.1055/a-0919-4388. Epub 2019 Jun 24.
9
Clinical and hormonal characteristics of patients with different types of hypophysitis: a single-center experience.不同类型垂体炎患者的临床和激素特征:单中心经验
Arch Endocrinol Metab. 2019 Feb;63(1):47-52. doi: 10.20945/2359-3997000000102.
10
Hypophysitis Outcome and Factors Predicting Responsiveness to Glucocorticoid Therapy: A Prospective and Double-Arm Study.垂体炎结局及预测糖皮质激素治疗反应的因素:一项前瞻性、双臂研究。
J Clin Endocrinol Metab. 2018 Oct 1;103(10):3877-3889. doi: 10.1210/jc.2018-01021.