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
ARTICLE HIGHLIGHTS
Case characteristics

A sixty-seven-year-old female presented with a progressive multi-organ involvement of IgG4-related disease (IgG4-RD) for over 19 years.

Clinical diagnosis

As a disorder with multiple organ involvement, IgG4-RD presents no specifically clinical manifestation for an early diagnosis, and the diagnosis largely relies on a combination of clinical manifestation, histology, imaging, and serology.

Differential diagnosis

IgG4-RD has a broad spectrum of clinical manifestations and it is important to differentially diagnosis it from various diseases of the involved organs, such as Mikuri’s disease, autoimmune pancreatitis, interstitial pneumonia and retroperitoneal fibrosis.

Laboratory diagnosis

A significantly increased serum IgG4 level (> 1350 mg/L) is a signal for serological diagnosis in IgG4-RD.

Imaging diagnosis

Computed tomography, magnetic resonance imaging or endoscopic retrograde cholangiopancreatography imaging showed a swollen feature or tumor-like structure in the involved organs.

Pathological diagnosis

IgG4-positive lymphocyte infiltration and sclerosis in the involved organs.

Treatment

The patient was given methylprednisolone 200 mg/d pulse therapy for 3 d, a large dose of gamma globulin (20 g/d) intravenous infusion for 3 d and taking oral prednisolone 30 mg/d for three weeks. The prednisolone was then gradually tapered.

Related reports

Patients with a clinically unclear cause of inflammation, swelling and refractory glands (such as the lacrimal gland, parotid gland and thyroid), rhinitis, pancreatitis, hypophysitis, and/or interstitial pneumonia should be considered for the possibility of IgG4-RD and recommended for plasma IgG4 test.

Term explanation

IgG4-RD is a systemic and clinical entity with autoimmune pathogenesis, which is characterized by high levels of circulating IgG4 and a dramatic response to steroid therapy.

Experiences and lessons

Since the clinical manifestations of IgG4-RD are commonly similar to features of other diseases, it is easily misdiagnosed and improperly treated. This case emphasizes a clinical importance of the early diagnosis and interventions for IgG4-RD. From a diagnostic standpoint, the early disease symptoms are generally mild with an involvement of one or two organs for a long time before they gradually develop into multiple organ involvements with life-threatening complications. Apart from clinical symptoms, concentrations of serum IgG4, IgE, ESR, CRP and other laboratory results may provide important diagnostic data and clues for early identity disease activities. From a therapeutic standpoint, patients with IgG4-RD respond well to steroid therapy, and/or immunosuppressive agents resulting in a significant remission of disease and improved patient conditions. Notably, the disease can easily recur after the steroid is reduced or withdrawn. To reduce the adverse effects of long-term steroid therapy, it is critical to give pulse therapy with high doses of steroids in combination with immunosuppressive agents during the active disease period of the disease in clinical settings.