Strainiene S, Sarlauskas L, Savlan I, Liakina V, Stundiene I, Valantinas J. Multi-organ IgG4-related disease continues to mislead clinicians: A case report and literature review. World J Clin Cases 2020; 8(15): 3267-3279 [PMID: 32874981 DOI: 10.12998/wjcc.v8.i15.3267]
Corresponding Author of This Article
Sandra Strainiene, MD, Doctor, Clinic of Gastroenterology, Nephrourology and Surgery, Centre of Hepatology, Gastroenterology and Dietetics, Institute of Clinical Medicine, Vilnius University, 3 Universiteto Street, Vilnius 01513, Lithuania. sandra.strainiene@santa.lt
Research Domain of This Article
Medicine, Research & Experimental
Article-Type of This Article
Case Report
Open-Access Policy of This Article
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/
Sandra Strainiene, Ilona Savlan, Valentina Liakina, Ieva Stundiene, Jonas Valantinas, Clinic of Gastroenterology, Nephrourology and Surgery, Centre of Hepatology, Gastroenterology and Dietetics, Institute of Clinical Medicine, Vilnius University, Vilnius 01513, Lithuania
Lukas Sarlauskas, Clinic of Internal Diseases, Family Medicine and Oncology, Centre of Internal Diseases, Institute of Clinical Medicine, Vilnius University, Vilnius 01513, Lithuania
Valentina Liakina, Department of Chemistry and Bioengineering, Faculty of Fundamental Science, Vilnius Gediminas Technical University, Vilnius 10223, Lithuania
Author contributions: Savlan I was the gastroenterologist who followed-up the patient; Strainiene S, Sarlauskas L and Savlan I performed patient's data extraction; Strainiene S and Sarlauskas L wrote the original manuscript and reviewed the literature; Savlan I, Liakina V and Stundiene I reviewed and edited the manuscript; Savlan I, Liakina V, Stundiene I and Valantinas J were responsible for the revision of the manuscript for the important intellectual content; all the authors issued the final approval for the version to be submitted and agreed to be accountable for all aspects of the work.
Informed consent statement: Informed written consent was obtained from the patient for the publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
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: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Sandra Strainiene, MD, Doctor, Clinic of Gastroenterology, Nephrourology and Surgery, Centre of Hepatology, Gastroenterology and Dietetics, Institute of Clinical Medicine, Vilnius University, 3 Universiteto Street, Vilnius 01513, Lithuania. sandra.strainiene@santa.lt
Received: April 15, 2020 Peer-review started: April 15, 2020 First decision: April 26, 2020 Revised: May 7, 2020 Accepted: July 15, 2020 Article in press: July 15, 2020 Published online: August 6, 2020 Processing time: 113 Days and 3.5 Hours
Abstract
BACKGROUND
Immunoglobulin G4-related disease (IgG4-RD) is a multisystemic mass forming immune-mediated disease that affects almost every organ and is a diagnostic challenge for every clinician. There is a lack of adequate epidemiological data worldwide, and evidence-based treatment recommendations are not yet established. We report the first case of IgG4-RD from Lithuania and the Baltic Sea region presented with thyroiditis, orbital myositis, orbitopathy, uveitis, scleritis, sialadenitis, autoimmune pancreatitis and prostatitis.
CASE SUMMARY
A 54-year-old Caucasian male was admitted to our tertiary Centre complaining of severe weight loss, diarrhoea, abdominal pain, salivary gland swelling, sicca symptoms and diplopia. On examination, bilateral palpable masses in the projection of major salivary glands, severe protrusion of the left eyeball and cachexia were noted. The patient was previously diagnosed with autoimmune thyroiditis and endocrine ophthalmopathy. The magnetic resonance imaging (MRI) of the head revealed enlarged extraocular muscles indicating orbital myositis. The biopsy from the salivary gland mass indicated sialadenitis. Abdominal MRI showed signs of autoimmune pancreatitis, and a serological test revealed the elevated serum IgG4 concentration. The patient was then diagnosed with IgG4-RD and successfully treated with prednisolone. There was a significant clinical, serological and radiological improvement after one month of treatment and no signs of relapse within twenty months. However, it took almost 18 years and the efforts of eight different medical specialists to establish the correct diagnosis.
CONCLUSION
A comprehensive approach to the patient is essential to improving the recognition of rare immune system conditions, such as IgG4-RD.
Core tip: Immunoglobulin G4-related disease (IgG4-RD) is a rare multisystemic immune-mediated disease that affects almost every organ and mimics various malignant, infectious or other inflammatory conditions. Therefore, the correct diagnosis and proper treatment are often delayed. We present the first clinical case of multi-organ IgG4-RD from Lithuania and the Baltic Sea region that reveals the importance of a comprehensive approach to the patient in order to achieve effective recognition of the rare immune system malfunction conditions such as IgG4-RD.
