Case Report
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 6, 2018; 6(13): 707-715
Published online Nov 6, 2018. doi: 10.12998/wjcc.v6.i13.707
Highlighting the importance of early diagnosis in progressive multi-organ involvement of IgG4-related disease: A case report and review of literature
Jing Xue, Xue-Mei Wang, Yan Li, Li Zhu, Xiao-Ming Liu, Juan Chen, Shu-Hong Chi
Jing Xue, Xue-Mei Wang, Shu-Hong Chi, Department of Rheumatology, General Hospital of Ningxia Medical University, Yinchuan 750004, Ningxia Hui Autonomous Region, China
Jing Xue, Xiao-Ming Liu, Institute of Human Stem Cell Research, General Hospital of Ningxia Medical University, Yinchuan 750004, Ningxia Hui Autonomous Region, China
Yan Li, Li Zhu, Department of Radiology, General Hospital of Ningxia Medical University, Yinchuan 750004, Ningxia Hui Autonomous Region, China
Juan Chen, Department of Pulmonary and Critical Care Medicine, General Hospital of Ningxia Medical University, Yinchuan 750004, Ningxia Hui Autonomous Region, China
Author contributions: Xue J collected patient’s clinical data, made a review of the literature and drafted the manuscript; Wang XM, Zhu L, Li Y and Chen J contributed to the acquisition, analysis and interpretation of histopathological findings; Liu XM revised the manuscript and Chi SH participated in collecting patient’s clinical data and critically revised the manuscript for intellectual contents; all authors read and approved the final manuscript.
Informed consent statement: Written informed consent was obtained from the patient for publication of this case.
Conflict-of-interest statement: The authors declare that they have no competing interests.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Shu-Hong Chi, MD, MSc, Associate Professor, Chief Doctor, Department of Rheumatology, General Hospital of Ningxia Medical University, 804 Shengli South Street, Xingqing District, Yinchuan 750004, Ningxia Hui Autonomous Region, China. chi794613@163.com
Telephone: +86-951-6744457
Received: July 31, 2018
Peer-review started: July 31, 2018
First decision: August 24, 2018
Revised: September 16, 2018
Accepted: October 11, 2018
Article in press: October 11, 2018
Published online: November 6, 2018
Processing time: 98 Days and 6.1 Hours
Abstract

IgG4-related disease (IgG4-RD) is an increasingly recognized pathological entity that tends to involve multiple organs with an elevated level of serum IgG4, which is easily misdiagnosed owing to sharing common clinical features with a variety of other diseases. Here, we report an interesting IgG4-RD case of a woman with progressive multi-organ involvement for over 19 years, started with swollen eyelids, dry eye and mouth, and polydipsia and hydruria. Imaging diagnosis revealed diffuse enlargement of the parotid glands, enlargement of the head of the pancreas, pulmonary infection and interstitial lung. Serological tests showed a remarkable elevation of the serum IgG4, and cytological analysis further revealed a large amount of lymphoplasmacytic infiltration into the focal lobule, and IgG4-positive cell infiltration in bladder mucosa. Therapeutically, the patient responded well to steroid therapy, and thus, she was diagnosed as IgG4-RD suspicious. This report highlights the importance of an early diagnosis in this autoimmune disease and suggests that patients with a clinically unclear cause of inflammation, swelling and refractory glands, rhinitis, pancreatitis, hypophysitis, and/or interstitial pneumonia should be considered for IgG4-RD. The plasma IgG4 level and lymphoplasmacytic infiltration may be useful indexes for screening, and a low dose of steroid maintaining therapy may offer benefits for patients with IgG4-RD.

Keywords: IgG4; IgG4-related disease; Autoimmune disease; Steroid therapy

Core tip: IgG4-related disease (IgG4-RD) is an easily misdiagnosed immune disorder with an elevated serum IgG4 and multiple organ involvements. Hence, it is important in early diagnosis for guiding clinical treatments. Here, we report an interesting case of a woman with progressive multi-organ involvement of IgG4-RD for 19 years. The 67-year-old woman has suffered from progressive diseases of multiple organs, including lacrimal glands, kidneys, parotid glands, submandibular glands, salivary glands, pituitary, pancreas and lung. Patients with IgG4-RD normally respond well to steroid therapy; however, the broad spectrum of manifestation with commonly clinical features of other diseases easily leads to misdiagnosis and improper treatment in clinical settings. Therefore, this report highlights the importance of early diagnosis in this autoimmune disease.