Abstract | OBJECTIVE:
Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized disease entity characterized by high-serum IgG4 concentration and IgG4-producing plasma cell production with fibrotic or sclerotic changes in affected organs. We aimed to clarify the roles of Epstein-Barr virus (EBV) in patients with IgG4-RDs. STUDY DESIGN AND SETTING: A retrospective clinical study at the Yamagata University School of Medicine, Yamagata, Japan. METHODS: The patient group consisted of four males and four females with an average age of 62 years (range: 48-73). Expression of IgG4, latent member protein 1, EBV nuclear antigens-2, and EBV-encoded RNA in affected salivary glands from patients with IgG4-RD was examined by using immunohistochemistry and in situ hybridization. The copy number of EBV DNA in the salivary glands was also investigated by real-time polymerase chain reaction. RESULTS: All patients had hard masses in the salivary or lacrimal glands, or both, bilaterally. Serum concentrations of IgG4 were elevated in all cases (mean 589.1, range 129-1750), and IgG4-positive plasmacytes were observed in the involved salivary glands. Four patients developed potentially life-threatening systemic involvement after initial salivary gland swelling. EBV-associated molecules (EBNA and EBER) were overexpressed in the affected salivary glands. The copy number of EBV DNA was significantly higher in patients with potentially life-threatening systemic involvement than in patients without systemic involvement (P < 0.05). CONCLUSION: These results suggest that the copy number of EBV DNA could be useful as diagnostic findings in IgG4-RD to predict potentially life-threatening systemic involvement. LEVEL OF EVIDENCE: 4.
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Authors | Takatoshi Furukawa, Yoshitaka Shimotai, Nobuo Ohta, Akihiro Ishida, Kazuya Kurakami, Hitoshi Suzuki, Mitsunori Yamakawa, Seiji Hongo, Seiji Kakehata |
Journal | The Laryngoscope
(Laryngoscope)
Vol. 125
Issue 9
Pg. 2079-84
(Sep 2015)
ISSN: 1531-4995 [Electronic] United States |
PMID | 26152762
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Copyright | © 2015 The American Laryngological, Rhinological and Otological Society, Inc. |
Chemical References |
- Antibodies, Anti-Idiotypic
- Immunoglobulin G
- RNA, Viral
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Topics |
- Aged
- Antibodies, Anti-Idiotypic
(immunology)
- Autoimmune Diseases
(epidemiology, immunology, virology)
- Epstein-Barr Virus Infections
(epidemiology, immunology, virology)
- Female
- Follow-Up Studies
- Herpesvirus 4, Human
(genetics)
- Humans
- Immunoglobulin G
(immunology)
- Immunohistochemistry
- In Situ Hybridization
- Incidence
- Japan
(epidemiology)
- Male
- Middle Aged
- RNA, Viral
(analysis)
- Real-Time Polymerase Chain Reaction
- Retrospective Studies
- Salivary Gland Diseases
(epidemiology, immunology, virology)
- Salivary Glands
(immunology, virology)
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