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IgG4 immunostaining and its implications in orbital inflammatory disease
Amanda J Wong1, Stephen R Planck2, Dongseok Choi3
1Casey Eye Institute, Oregon Health & Science University, Portland, Oregon, United States of America.
Plos One
|October 11, 2014
Summary
Immunoglobulin G4 (IgG4)-related disease is often found in the orbit. This study found IgG4-positive plasma cells are common in orbital tissues of patients with sarcoidosis, granulomatosis with polyangiitis (GPA), or nonspecific orbital inflammatory disease (NSOI).
Area of Science:
- Ophthalmology
- Immunology
- Pathology
Background:
- Immunoglobulin G4 (IgG4)-related disease is an emerging condition affecting orbital tissues.
- Understanding IgG4 immunostaining is crucial for diagnosing orbital inflammatory diseases.
Purpose of the Study:
- To analyze gene expression and IgG4 immunostaining prevalence in orbital inflammatory diseases.
- To clarify the role of IgG4 in orbital inflammation.
Main Methods:
- Collected orbital biopsies from 108 subjects (including controls, NSOI, TED, sarcoidosis, GPA).
- Performed IgG4 immunostaining on lacrimal gland and orbital adipose tissue.
- Quantified RNA transcripts using Affymetrix arrays.
Main Results:
- No significant IgG4 staining in healthy controls or thyroid eye disease (TED).
- IgG4 staining detected in 11-39% of other subjects, common in GPA, less so in sarcoidosis and NSOI.
- IgG4+ cells correlated with lacrimal gland inflammation and increased B cell-related gene expression.
Conclusions:
- IgG4-positive plasma cells are prevalent in orbital tissues of patients with sarcoidosis, GPA, or NSOI.
- IgG4 staining correlates with increased lacrimal gland inflammation and specific gene expression patterns.

