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韦格纳肉芽肿病累及垂体的罕见表现:病例报告及文献复习

Rare presentation of Wegener's granulomatosis in the pituitary gland: Case report and literature review.

作者信息

Baird Samantha M, Pratap Upasna, McLean Catriona, Law Candice P, Maartens Nicholas

机构信息

Department of Ear Nose and Throat Surgery, The Alfred Hospital, 55 Commercial Road, Prahran, VIC 3181, Australia.

Department of Anatomical Pathology, The Alfred Hospital, 55 Commercial Road, Prahran, VIC 3181, Australia.

出版信息

Int J Surg Case Rep. 2017;33:24-26. doi: 10.1016/j.ijscr.2017.02.014. Epub 2017 Feb 20.

Abstract

INTRODUCTION

Wegener's granulomatosis (WG) is a systemic vasculitis that can affect a variety of organs including ear, nose and throat, lungs and kidneys. However WG is unusual in the pituitary and rare in the central nervous system.

PRESENTATION OF CASE

A 56-year-old male with likely WG presented with polyuria and polydipsia despite six months of conservative medical management. MRI scanning revealed an enlarging heterogeneously enhancing pituitary gland. Following endoscopic transsphenoidal pituitary biopsy and debulking, final tissue pathology was diagnostic for WG in the pituitary gland.

DISCUSSION

Diagnosis remains difficult but most patients present with central diabetes insipidus (CDI) as well as varying degrees of hypopituitarism on a background of disease activity in other organs. Clinical judgment needs to balance the need for invasive surgical tissue diagnosis with increasing immunosuppressive therapy.

CONCLUSION

It is important to consider this rare complication of WG to ensure timely diagnosis and management.

摘要

引言

韦格纳肉芽肿(WG)是一种系统性血管炎,可累及包括耳、鼻、喉、肺和肾脏在内的多种器官。然而,WG累及垂体并不常见,累及中枢神经系统则更为罕见。

病例介绍

一名56岁男性,疑似患有WG,尽管经过了6个月的保守药物治疗,仍出现多尿和烦渴症状。MRI扫描显示垂体增大,强化不均匀。在内镜经蝶窦垂体活检及减压术后,最终组织病理学诊断为垂体WG。

讨论

诊断仍然困难,但大多数患者在其他器官疾病活动的背景下,会出现中枢性尿崩症(CDI)以及不同程度的垂体功能减退。临床判断需要在侵入性手术组织诊断的必要性与增加免疫抑制治疗之间取得平衡。

结论

认识到WG的这种罕见并发症很重要,以确保及时诊断和治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/deb0/5338910/980293cf5cac/gr1.jpg

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