Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 26, 2022; 10(18): 6269-6276
Published online Jun 26, 2022. doi: 10.12998/wjcc.v10.i18.6269
Clivus-involved immunoglobulin G4 related hypertrophic pachymeningitis mimicking meningioma: A case report
Yang Yu, Liang Lv, Sen-Lin Yin, Cheng Chen, Shu Jiang, Pei-Zhi Zhou
Yang Yu, Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
Liang Lv, Sen-Lin Yin, Cheng Chen, Shu Jiang, Pei-Zhi Zhou, Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
Author contributions: Yu Y collected the data, contacted with the patient, and wrote the manuscript; Lv L wrote and revised the manuscript; Chen C and Yin SL made the revision to the primary manuscript; Jiang S and Zhou PZ supervised the whole work and made the operation.
Supported by 1·3·5 Project for Disciplines of Excellence–Clinical Research Incubation Project, West China Hospital, Sichuan University, No. 2019HXFH018.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.
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: Pei-Zhi Zhou, MD, Associate Professor, Surgeon, Department of Neurosurgery, West China Hospital, Sichuan University, No. 37 Guoxue Alley, Chengdu 610041, Sichuan Province, China. peizhizhou@126.com
Received: December 13, 2021
Peer-review started: December 13, 2021
First decision: February 14, 2022
Revised: February 23, 2022
Accepted: April 22, 2022
Article in press: April 22, 2022
Published online: June 26, 2022
Processing time: 185 Days and 11.1 Hours
Abstract
BACKGROUND

Immunoglobulin G4 related disease (IgG4-RD) is a fibroinflammatory disease with markedly elevated serum IgG4 levels and fibrous tissue proliferation, accompanied by numerous plasma cells. IgG4 related hypertrophic pachymeningitis (IgG4-RHP) is relatively rare and indistinguishable from other phymatoid diseases before the operation. The risk of long-term immunosuppression needs to be balanced with disease activity.

CASE SUMMARY

A 40-year-old man presented with headache and bilateral abducent paralysis. He was also diagnosed with pulmonary tuberculosis 10 years ago and was on regular treatment for the same. Before the operation and steroid therapy, the patient was suspected of having tubercular meningitis at a local hospital. A clivus lesion was found via brain magnetic resonance imaging (MRI) at this presentation. He was preliminarily diagnosed with meningioma and underwent Gamma Knife Surgery. Transnasal endoscopic resection was performed to treat deterioration of nerve function. Postoperative pathologic examination suggested IgG4-RD. Moreover, the serum IgG4 was elevated at 1.90 g/L (reference range: 0.035-1.500 g/L). After steroid therapy for 2 mo, the lesion size diminished on MRI, and the function of bilateral abducent nerves recovered.

CONCLUSION

IgG4-RHP is relatively rare and indistinguishable before the operation. Elevated serum IgG4 levels and imaging examination help in the diagnosis of IgG4-RHP. Surgery is necessary when lesions progress and patients start to develop cranial nerve function deficit.

Keywords: Immunoglobulin G4 related disease; Hypertrophic pachymeningitis; Immunoglobulin G4 related hypertrophic pachymeningitis; Clivus; Case report

Core Tip: Immunoglobulin G4 related disease (IgG4-RD) is a fibroinflammatory disease with markedly elevated serum IgG4 levels and fibrous tissue proliferation, accompanied by numerous plasma cells. It is known to affect multiple organs. IgG4-related hypertrophic pachymeningitis (IgG4-RHP) is relatively rare and indistinguishable from IgG4-RD before the operation. Herein, we present a rare case of IgG4-RHP with intact magnetic resonance imaging and pathologic images. The case highlighted the differential diagnosis with other phymatoid lesions such as meningioma, fungal infection, and tuberculosis and the importance of comprehensive multidisciplinary treatment. Surgery becomes necessary when lesions progress and patients start to develop cranial nerve function deficit.