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Mod Pathol. 2018 Jun;31(6):890-899. doi: 10.1038/s41379-018-0036-4. Epub 2018 Feb 12.

Glucocorticoid receptor expression in resident and hematopoietic cells in IgG4-related disease.

Author information

1
TMK Project, Medical Innovation Center, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, Japan.
2
Research Unit/Innovative Medical Science, Sohyaku Innovative Research Division, Mitsubishi Tanabe Pharma Corporation, Toda, Saitama, Japan.
3
Department of Nephrology, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, Japan.
4
Department of Nephrology, Japanese Red Cross Otsu Hospital, Otsu, Shiga, Japan.
5
Divisions of Nephrology, Department of Internal Medicine, Nippon Medical School, Tokyo, Japan.
6
Division of Rheumatology Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Ishikawa, Japan.
7
Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, Japan.
8
Department of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, Japan.
9
Department of Urology, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, Japan.
10
Department of Otolaryngology-Head and Neck Surgery, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, Japan.
11
Department of Oral and Maxillofacial Surgery, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, Japan.
12
Department of Otolaryngology-Head and Neck Surgery, Kanazawa University Graduate School of Medicine, Kanazawa, Ishikawa, Japan.
13
Division of Nephrology and Rheumatology, Department of Internal Medicine, Faculty of Medicine, Fukuoka University, Fukuoka, Fukuoka, Japan.
14
Sanko Clinic, Fukuoka, Fukuoka, Japan.
15
Department of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Kyoto, Japan.
16
Department of Nephrology, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, Japan. motoy@kuhp.kyoto-u.ac.jp.

Abstract

Immunoglobulin G4-related disease is a rare immune-mediated disease characterized by the infiltration of IgG4-positive plasma cells and unique storiform fibrosis of multiple organs. The majority of IgG4-related disease patients respond to glucocorticoids, yet the precise mechanism of their action remains unclear. Pathological sections of the submaxillary gland, kidney, and retroperitoneum from 20 patients in total diagnosed with IgG4-related disease were analyzed for glucocorticoid receptor expression and the cell types expressing glucocorticoid receptor. Strong and abundant expression of glucocorticoid receptor was observed in the submaxillary gland, kidney, and retroperitoneum of IgG4-related disease patients, while glucocorticoid receptor was rarely or only faintly observed in the submaxillary gland of patients with Sjögren's syndrome, radicular cysts and sialolithiasis or in the healthy kidney. Glucocorticoid receptor was mainly expressed in fibro/myofibroblasts, CD4-positive T cells and IgG4-positive plasma cells in the submandibular glands and kidneys of IgG4-related disease patients. The abundant expression of glucocorticoid receptor in various types of cells, including resident fibro/myofibroblasts in IgG4-related disease patients might provide clues to the mechanism of steroid responsiveness in IgG4-related disease patients.

PMID:
29434340
DOI:
10.1038/s41379-018-0036-4
[Indexed for MEDLINE]
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