Observational Study
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 6, 2019; 7(19): 2942-2952
Published online Oct 6, 2019. doi: 10.12998/wjcc.v7.i19.2942
Association of stiff-person syndrome with autoimmune endocrine diseases
Yi-Yin Lee, I-Wen Chen, Szu-Tah Chen, Chih-Ching Wang
Yi-Yin Lee, I-Wen Chen, Szu-Tah Chen, Chih-Ching Wang, Department of Internal Medicine, Division of Endocrinology and Metabolism, Chang Gung Memorial Hospital, Taoyuan City 333, Taiwan
Author contributions: Lee YY wrote the manuscript, analyzed the data, and contributed to discussion; Wang CC analyzed the data and contributed to discussion; Chen IW and Chen ST contributed to discussion and reviewed/edited the manuscript; Wang CC is the guarantor of this work, has full access to all the data in the study, and takes responsibility for the integrity of the data.
Institutional review board statement: The study was reviewed and approved by the Chang Gung Memorial Hospital Institutional Review Board.
Informed consent statement: This study was approved by the Institutional Review Board of Chang Gung Memorial Hospital, and informed consent was not required because that data was drawn from observing behavior and did not contain identifiable information.
Conflict-of-interest statement: The authors report no relevant conflicts of interest.
Data sharing statement: The authors confirm that the data supporting the findings of this study are available within the article.
STROBE statement: The authors have read the STROBE Statement-checklist of items, and the manuscript was prepared and revised according to the STROBE Statement-checklist of items.
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 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/
Corresponding author: Chih-Ching Wang, MD, Attending Doctor, Endocrinologist, Department of Internal Medicine, Division of Endocrinology and Metabolism, Chang Gung Memorial Hospital, No. 5, Fuxing Street, Guishan District, Taoyuan City 333, Taiwan. p122020223@cgmh.org.tw
Telephone: +886-3-3281200 Fax: +886-3-3288257
Received: June 27, 2019
Peer-review started: June 29, 2019
First decision: July 31, 2019
Revised: August 14, 2019
Accepted: August 27, 2019
Article in press: August 27, 2019
Published online: October 6, 2019
ARTICLE HIGHLIGHTS
Research background

Glutamic acid decarboxylase (GAD) is known to synthesize the inhibitory neurotransmitter of γ-aminobutyric acid, and it is also found in the β-cells in the pancreas. Clinically, anti-GAD Ab is associated with stiff-person syndrome (SPS), type 1 diabetes mellitus (T1DM), and other autoimmune diseases.

Research motivation

There is still a plausible and unclear mechanism in SPS with autoantibodies and related other autoimmune diseases.

Research objectives

To investigate the link of autoimmune endocrine disorders with anti-GAD Ab in SPS subjects.

Research methods

Patients with SPS collected from January 2001 to June 2018 were retrospectively analyzed. Anti-GAD antibodies were measured using radioimmunoassay and enzyme-linked immunosorbent assay to determine the diagnosis of and association with other autoimmune diseases.

Research results

Of the 14 patients, 12 (85.7%) were diagnosed with classic SPS and 2 (14.3%) with stiff limb syndrome (SLS). Among nine classic SPS patients who underwent the anti-GAD Ab test, three were anti-GAD Ab seropositive and each of these three patients also had T1DM, latent autoimmune diabetes in adults, and autoimmune thyroid disease. In contrast, other rare autoimmune diseases co-existed in six anti-GAD Ab seronegative SPS patients. None of the SLS patients had additional autoimmune disease.

Research conclusions

The presence of typical clinical symptoms and anti-GAD autoantibody are not only important clues for diagnosis of SPS but also for early detection of this disease and prediction of the association with other autoimmune diseases.

Research perspectives

This article reflects that anti-GAD autoantibody may demonstrate the diagnostic accuracy of SPS, although there is a lack of large sample size and unclear mechanism due to rarity. SPS is associated with other autoimmune diseases like T1DM and malignancy, which can cause morbidity and mortality. Further investigation of the link between SPS and T1DM could predict DM and prevent significant disabilities.