Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Sep 16, 2022; 10(26): 9440-9446
Published online Sep 16, 2022. doi: 10.12998/wjcc.v10.i26.9440
Imaging findings of immunoglobin G4-related hypophysitis: A case report
Kun Lv, Xin Cao, Dao-Ying Geng, Jun Zhang
Kun Lv, Xin Cao, Dao-Ying Geng, Jun Zhang, Department of Radiology, Huashan Hospital, Fudan University, Shanghai 200040, China
Kun Lv, Xin Cao, Dao-Ying Geng, Jun Zhang, Institute of Functional and Molecular Medical Imaging, Fudan University, Shanghai 200040, China
Kun Lv, Xin Cao, Dao-Ying Geng, Jun Zhang, Center for Shanghai Intelligent Imaging for Critical Brain Diseases Engineering and Technology Research, Science and Technology Commission of Shanghai Municipality, Shanghai 200003, China
Kun Lv, Xin Cao, Dao-Ying Geng, Jun Zhang, Institute of Intelligent Imaging Phenomics, International Human Phenome Institutes (Shanghai), Shanghai 200433, China
Author contributions: Geng DY designed the outline of the case report; Lv K wrote the first draft of the manuscript; Cao X edited the manuscript for important intellectual content; Zhang J and Lv K revised and edited the final version of the manuscript; all authors contributed to this article and approved the submitted version.
Supported by National Key R&D Program of China, No. 2019YFC0120602; Clinical Research Plan of SHDC, No. SHDC2020CR3020A; Research Startup Fund of Huashan Hospital, Fudan University, No. 2021QD035; Shanghai Municipal Commission of Science and Technology, No. 22S31905300; and Greater Bay Area Institute of Precision Medicine (Guangzhou), No. KCH2310094.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Dao-Ying Geng, MD, PhD, Chief Doctor, Department of Radiology, Huashan Hospital, Fudan University, No. 12 Wulumuqi Middle Road, Shanghai 200040, China. gdy_2019@163.com
Received: April 20, 2022
Peer-review started: April 20, 2022
First decision: June 16, 2022
Revised: July 4, 2022
Accepted: August 16, 2022
Article in press: August 16, 2022
Published online: September 16, 2022
Abstract
BACKGROUND

Immunoglobin G4 (IgG4)-related hypophysitis (IgG4-RH) is a rare form of IgG4-related disease (IgG4-RD), which often manifests as a single organ disease and is easily misdiagnosed as a pituitary tumor clinically and by imaging. There are few reports of imaging findings of IgG4-RH. Therefore, we describe a case of IgG4-RH, which mimicked a pituitary macroadenoma, that was detected by computed tomography (CT) and magnetic resonance imaging (MRI), and review the previous literature in order to further the understanding of IgG4-RH.

CASE SUMMARY

A 47-year-old man presented with a history of blurred vision for more than 2 mo, without other symptoms. A preoperative unenhanced CT scan revealed a slightly hyperdense mass in the sellar region measuring 2.5 cm × 2.3 cm × 1.8 cm, with a CT value of 45 HU. T1-weighted imaging (T1WI) and T2-weighted imaging showed iso-hypointensity, and gadolinium contrast-enhanced T1WI showed obvious homogeneous enhancement. The MRI revealed involvement of the pituitary gland and stalk. Preoperative laboratory tests revealed abnormal pituitary hormone levels, including an increased prolactin level, and decreased levels of insulin-like growth factor, dehydroepiandrosterone, and testosterone. The lesion was surgically resected. Postoperative histopathological examination of a tissue sample and an elevated serum IgG4 level confirmed the diagnosis of IgG4-RH. The patient was treated with cortisone acetate postoperatively and made a good recovery without developing any neurological deficit.

CONCLUSION

An elevated serum IgG4 concentration is the main clue for diagnosis of IgG4-RD. Imaging combined with laboratory testing is useful for preoperative diagnosis of IgG4-RH.

Keywords: Immunoglobin G4-related disease, Pituitary, Macroadenoma, Inflammation, Hypophysitis, Case report

Core Tip: Immunoglobin G4 (IgG4)-related hypophysitis (IgG4-RH) is a rare form of IgG4-related disease, which is easily misdiagnosed as a pituitary tumor. IgG4-RH commonly occurs in middle-aged and older men and is characterized by hypointensity on T2-weighted imaging and homogeneous and obvious enhancement on gadolinium contrast-enhanced T1-weighted imaging, accompanied by hypopituitarism. The imaging findings help to differentiate it from pituitary tumors. An elevated serum IgG4 level is the main clue to the diagnosis of IgG4-RH, and imaging, histopathology, or response to glucocorticoid therapy can be used to confirm the diagnosis.